Moderate Pulmonary Hypertension Research Articles

Pregnancy conditions and outcomes of Chinese women with mild, moderate and severe pulmonary arterial hypertension.

Pregnancy is normally contraindicated in pulmonary arterial hypertension (PAH). Thanks to medical advances, the prognosis for pregnancy in patients with PAH has improved. The aim of our study was to investigate pregnancy conditions and outcomes in patients with mild, moderate and severe PAH. We searched PubMed, Embase, CNKI, Wanfang and Weipu databases for studies published before May 2024. Data from 29 included studies from 1898 references were pooled and analyzed. We calculated the rates for each group as well as the risk ratio (RR) and 95% confidence interval (CI) between pairwise. There was no statistical difference in maternal and neonatal survival between the mild and moderate groups. Maternal survival in the mild, moderate and severe groups was 100.0%, 99.7% and 88.8%, respectively, and neonatal survival was 100.0%, 99.7% and 96.0%, respectively. The incidence of NYHA class III-IV, pregnancy loss, intensive care unit (ICU) admission, fetal growth restriction, and neonatal asphyxia was lowest in patients with mild PAH and highest in patients with severe PAH (P < 0.001). The incidence of vaginal deliveries and term pregnancies was highest in the mild group and lowest in the severe group (P < 0.001). In conclusion, pregnant women with mild PAH can safely deliver a newborn. Given similar survival rates but greater economic and medical burdens, caution is advised in the moderate group. Pregnancy in the severe group is considered contraindicated.

Atrial flutter/fibrillation in adult congenital heart disease: Insights from a large Asian cohort

BackgroundThe incidence of atrial fibrillation/atrial flutter (AF/AFL) in general population is lower in Asia compared to Western countries. It is unclear whether a similar trend exists among adults with congenital heart disease (ACHD). We determine the profile, risk factors, and impact of AF/AFL in an Asian ACHD cohort. MethodsWe included all ACHD patients diagnosed in an Asia tertiary care center between 2007 and 2018, analyzing AF (sustained and paroxysmal AF) and AFL, collectively./Purpose. ResultsThe study encompassed 4391 patients (55.9% women), with 81% having simple, 16.3% moderate and 2.8% severe CHD. AF/AFL was observed in 6.7% of the patients, with 54.6% having paroxysmal AF, 27.3% sustained AF, and 18.1% AFL. Incidence of AF/AFL increased with age and was higher in patients with pulmonary hypertension (PH), severe CHD, and metabolic syndrome. We found a progressive trend in the onset age of arrhythmia: AFL at a younger age, followed by paroxysmal and sustained AF. Risk factors for AF/AFL included severe and moderate CHD, PH, previous interventions, and male sex (odds ratio 11.2 and 3.15, 2.03, 1.75, and 1.71, respectively). When stratifying by CHD severity, PH and male sex were significant risk factors in simple CHD, while only PH in severe CHD. Patients with AF/AFL had a significantly lower major adverse cardiovascular events-free survival rate. ConclusionsThis large ACHD cohort from Asia exhibited a high incidence of AF/AFL, similar to Western reports. The risk of AF/AFL was primarily associated with hemodynamic factors such as PH and CHD severity.

Complete dislodgement of a mechanical valve prosthesis: A rare but potentially life-threatening event: a case report

Rationale: Complete dislodgement of a mechanical valve is extremely uncommon as a long-term issue after getting a substitute mitral valve, and this report details an incident of complete detachment of a mechanical valve. Patient concerns: A 50-year-old woman, who underwent mitral mechanical valve replacement 2 decades earlier at another facility, was urgently admitted due to sudden cardiogenic shock. Diagnoses: Transthoracic echocardiograms revealed severe malfunction of the mitral valve prosthesis, characterized by significant mitral regurgitation and moderate pulmonary hypertension. Following the insertion of extracorporeal membrane oxygenation and an intra-aortic balloon pump, the hemodynamics stabilized. Coronary angiography displayed the prosthetic mitral valve ring and leaflet floating in the left atrium, as confirmed by preoperative real-time 3-dimensional transesophageal echocardiography. A complete separation of the prosthetic ring and leaflet from the suture ring was observed. Interventions: The patient promptly underwent bioprosthetic mitral valve replacement. Outcomes: The patient’s postoperative course was uneventful, leading to discharge in good condition. Lessons: A crucial aspect is comprehending the structure of the prosthetic valve itself. The use of transthoracic echocardiography and real-time 3-dimensional transesophageal echocardiography provides additional structural and functional details, enhancing support for potential life-saving interventions. Echocardiography plays a significant role in evaluating the morphology and function of prosthetic valves.

Reappraisal of the Concept and Implications of Pulmonary Hypertension in Degenerative Mitral Regurgitation

BackgroundEuropean and U.S. clinical guidelines diverge regarding pulmonary hypertension (PHTN) in degenerative mitral regurgitation (DMR). Gaps in knowledge underpinning these divergences affect risk assessment and management recommendations attached to systolic pulmonary pressure (SPAP) in DMR. ObjectivesThis study sought to define PHTN links to DMR severity, prognostic thresholds, and independent outcome impact in a large quantitative DMR registry. MethodsThis study gathered a large multicentric registry of consecutive patients with isolated moderate-to-severe DMR, with DMR and SPAP quantified prospectively at diagnosis. ResultsIn 3,712 patients (67 ± 15 years, 36% women) with ≥ moderate-to-severe DMR, effective regurgitant orifice (ERO) was 0.42 ± 0.19 cm2, regurgitant volume 66 ± 327 mL/beat and SPAP 41 ± 16 mm Hg. Spline-curve analysis showed excess mortality under medical management emerging around SPAP 35 mm Hg and doubling around SPAP 50 mm Hg. Accordingly, severe pulmonary hypertension (sPHTN) (SPAP ≥50 mm Hg) was detected in 916 patients, moderate pulmonary hypertension (mPHTN) (SPAP 35-49 mm Hg) in 1,128, and no-PHTN (SPAP <35 mm Hg) in 1,668. Whereas SPAP was strongly associated with DMR-ERO, nevertheless excess mortality with sPHTN (adjusted HR: 1.65; 95% CI: 1.24-2.20) and mPHTN (adjusted HR: 1.44; 95% CI: 1.11-1.85; both P ≤ 0.005) was observed independently of ERO and all baseline characteristics and in all patient subsets. Nested models demonstrated incremental prognostic value of mPHTN and sPHTN (all P < 0.0001). Despite higher operative risk with mPHTN and sPHTN, DMR surgical correction was followed by higher survival in all PHTN ranges with strong survival benefit of early surgery (<3 months). Postoperatively, excess mortality was abolished (P ≥ 0.30) in mPHTN, but only abated in sPHTN. ConclusionsThis large international registry, with prospectively quantified DMR and SPAP, demonstrates a Doppler-defined PHTN impact on mortality, independent of DMR severity. Crucially, it defines objectively the new and frequent mPHTN range, independently linked to excess mortality under medical management, which is abolished by DMR correction. Thus, at DMR diagnosis, Doppler-SPAP measurement defining these new PHTN ranges, is crucial to guiding DMR management.

Long-term outcomes of repair in children with total anomalous pulmonary venous connection.

The clinical data of patients with total anomalous pulmonary venous connection who underwent repair in our centre in the past 13 years were reviewed. In this study, we systemically reviewed our experience in the optimal surgical strategy for patients with total anomalous pulmonary venous connection, aiming to provide evidence for clinical decision-making. From January 1, 2009, to December 31, 2021, 122 patients undergoing surgical treatment for total anomalous pulmonary venous connection in our hospital were enrolled. Among them, 18 patients with single ventricle repair were excluded from the study. Multivariate analysis was used to determine the risk factors for early and late death and the risk factors for pulmonary vein obstruction. There were 64 males and 40 females. The median age at surgery was 107 days (range, 25 days-788 days), the median weight at surgery was 4.8 kg (range, 3 kg-22 kg), and the median follow-up was 59 months (range, 0-150 months). Seven patients died early after surgery and six died late after discharge. Multivariable analysis indicated that prolonged cardiopulmonary bypass time was the only independent risk factor for early postoperative mortality. Multivariate analysis did not identify risk factors for late death. Emergency surgery, preoperative moderate and severe pulmonary hypertension, and prolonged cardiopulmonary bypass time were independent risk factors for postoperative pulmonary vein obstruction. Early and long-term late outcomes of repair in patients with total anomalous pulmonary venous connection have been encouraging. Postoperative pulmonary vein obstruction remains a major problem for specialists worldwide. Pulmonary vein obstruction should be considered in children with preoperative emergency surgery, moderate to severe pulmonary hypertension and prolonged cardiopulmonary bypass time, and regular follow-up is necessary.

Correlating Severity of Pulmonary Hypertension by Echocardiogram with Mortality in Premature Infants with Bronchopulmonary Dysplasia.

Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth. Infants with BPD are at increased risk for pulmonary hypertension (PH). Cardiac catheterization is the gold standard for diagnosing PH, but cardiac catheterization is challenging to perform in small, sick, premature infants. The utility of echocardiography for diagnosing PH and predicting outcomes in extremely premature infants has not been clearly defined. Therefore, we sought to use predefined criteria to diagnose PH by echocardiogram and relate PH severity to mortality in extremely premature infants with BPD. Echocardiograms from 46 infants born ≤28 weeks' postmenstrual age with a diagnosis of BPD were assessed for PH by three pediatric cardiologists using predefined criteria, and survival times among categories of PH patients were compared. A total of 458 echocardiograms were reviewed, and 15 (33%) patients were found to have at least moderate PH. Patients with at least moderate PH had similar demographic characteristics to those with no/mild PH. Ninety percent of infants without moderate to severe PH survived to hospital discharge, compared with 67% of infants with at least moderate PH (p = 0.048). Patients with severe PH had decreased survival to hospital discharge (38%) compared with moderate (100%) and no/mild PH (90%) groups. Kaplan-Meier survival curves also differed among PH severity groups (Wilcoxon p < 0.001). Using predefined criteria for PH, premature infants with BPD can be stratified into PH severity categories. Patients diagnosed with severe PH by echocardiogram have significantly reduced survival. · A composite score definition of PH by echocardiogram showed high inter- and intrarater reliability.. · Infants with severe PH by echocardiogram had decreased survival rates.. · Early diagnosis of PH by echocardiogram dictates treatment which may improve outcomes..

Exercise-Induced Pulmonary Hypertension in Long-Term Survivors of Congenital Diaphragmatic Hernia

ObjectiveTo determine the prevalence of exercise-induced pulmonary hypertension (PH) among long-survivors of congenital diaphragmatic hernia (CDH) repair. Study designThis is a single-center, retrospective cohort study of CDH survivors who underwent exercise stress echocardiography (ESE) at Boston Children’s Hospital from January 2006 to June 2020. PH severity was assessed by echocardiogram at baseline and after exercise. Patients were categorized by right ventricular systolic pressure (RVSP) after exercise: Group 1 - no or mild PH; and Group 2 - moderate or severe PH (RVSP ≥ 60 mmHg or ≥ ½ systemic blood pressure). ResultsEighty-four patients with CDH underwent 173 ESE with median age 8.1 (4.8 – 19.1) years at first ESE. Sixty-four patients were classified as Group 1, 11 as Group 2, and 9 had indeterminate RVSP with ESE. Moderate to severe PH after exercise was found in 8 (10%) patients with no or mild PH at rest. Exercise-induced PH was associated with larger CDH defect size, patch repair, use of ECMO, supplemental oxygen at discharge, and higher WHO functional class. Higher VE/VCO2 slope, lower peak oxygen saturation, and lower percent predicted FEV1 and FEV1/FVC ratio were associated with Group 2 classification. ESE changed management in 9/11 Group 2 patients. PH was confirmed in all 5 Group 2 patients undergoing cardiac catheterization after ESE. ConclusionsAmong long-term CDH survivors, 10% had moderate-severe exercise-induced PH on ESE, indicating ongoing pulmonary vascular abnormalities. Further studies are needed to optimally define PH screening and treatment for patients with repaired CDH.

Pulmonary Artery Hypertension: Fifty Years Following Pneumonectomy in Infancy.

Pulmonary hypertension (PH) may be the result of many different pathological processes. PH is a rare but recognized vascular complication following major lung resection. We describe the diagnosis and management of moderate PH resulting more than 50 years in a patient who underwent a total unilateral pneumonectomy in infancy. Unfortunately, patients who undergo pneumonectomy will likely go on to develop PH and their functional status will be greatly impacted. In the case presented, we report on a patient whose PH and symptoms improved following off-label WHO group 1 treatment.

Cardiopulmonary Involvement in Patients with Systemic Sclerosis: A Cross-sectional Study

Introduction: One of the most challenging aspects of systemic sclerosis is the potential involvement of the cardiopulmonary system, which can lead to significant morbidity and mortality if not detected and managed promptly. Aim: To assess the clinical characteristics and prevalence of specific cardiopulmonary involvement and to investigate its association with disease duration in patients with systemic sclerosis from South India. Materials and Methods: This cross-sectional, observational study was conducted at the Department of General Medicine, Government Medical College, Thrissur, India, between January 1, 2020, and December 31, 2020. Forty patients with systemic sclerosis were included according to the European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology classification criteria. Skin thickening was evaluated using the modified Rodnan Skin Score (mRSS). Investigations such as chest X-ray, echocardiogram, and antibody profiling were conducted to evaluate cardiopulmonary involvement. The data were analysed using Statistical Package for Social Sciences (SPSS) version 23.0, and the Chi-square test was used to compare qualitative variables between groups. Results: Sixteen patients (40%) had an mRSS score between 11 and 20, followed by 14 patients (35%) with mRSS scores between 21 and 30. The prevalence of Interstitial Lung Disease (ILD) was 50%. The echocardiogram findings showed mitral regurgitation and moderate Pulmonary Arterial Hypertension (PAH) in 30% (n=12) and 25% (n=10) of patients, respectively. High-Resolution Computed Tomography (HRCT) and pulmonary function tests were normal in 10 patients (25%). The duration of the disease was three to eight years in 14 patients and eight to 12 years in 12 patients. Both ILD and PAH were significantly associated with the duration of the disease (p&lt;0.05). Antiscleroderma-70 and anti-centromere were the most common antibodies present in patients with ILD and PAH, respectively. Conclusion: The study indicated a high prevalence of PAH and ILD in patients with systemic sclerosis. Moreover, both ILD and PAH were significantly associated with the duration of the underlying disease, with longer disease duration being associated with a higher likelihood of these conditions.

Cardiovascular Consequences of Sickle Cell Disease.

Sickle cell disease is an inherited blood disorder which can lead to severe complications, particularly in the cardiovascular and respiratory systems, potentially resulting in arrhythmias, pulmonary hypertension (PH), and cardiomegaly. This study aims to investigate the risk of PH and arrhythmias in adult SCD patients. Retrospective analysis of medical records from King Abdulaziz University Hospital (KAUH) for patients with SCD aged 15 and above between 2009 and 2021. The study included 517 patients, with echocardiograms and electrocardiograms assessed according to the European Society of Cardiology/the European Respiratory Society (ESC/ERS) guidelines for categorizing PH risk (low, moderate, high) and detecting arrhythmias. Data analysis employed the Statistical Package for the Social Sciences (SPSS), utilizing quantitative and qualitative data representation. Multivariate logistic regression identified independent risk factors with odds ratios at a 95% confidence interval (CI). Among participants, 50.3% were male, with a total sample average age of 34.45 ± 9.28 years. Results indicated that 1.4% of patients experienced arrhythmias, 3.7% had a moderate PH risk, and 3.3% were classified as high PH risk. Logistic regression revealed significant independent risk factors for PH and arrhythmia in patients with SCD, with chronic kidney disease (CKD) carrying the highest odds (26.4 times higher odds of PH and 15.36 times higher odds of arrhythmias). Patients with SCD are at risk for developing PH and various arrhythmias but are often underdiagnosed. Key risk factors for PH included CKD, liver cirrhosis, and pre-existing cardiac conditions. Arrhythmias were significantly associated with CKD and pre-existing cardiac conditions. To mitigate these risks, we recommend involving a multidisciplinary healthcare team in the care of adult patients with SCD. Future prospective studies are advised for early detection of PH and arrhythmias in hemoglobinopathy patients, potentially reducing mortality.

Left ventricular eccentricity index to assess precapillary pulmonary hypertension in dogs

IntroductionInterventricular septal flattening, frequently present in pulmonary hypertension (PH) can be quantified by the left ventricular eccentricity index (EI) measured at end-diastole (EId), end-systole (EIs) and at maximal septal flattening (EIm). In humans, EI correlates with invasive pulmonary arterial pressure.The aim of this study was to evaluate if EI correlates with parameters of right heart remodeling (RHR) and if EI is a quantitative marker of PH in dogs. Materials and methodsLeft ventricular eccentricity indices were retrospectively measured in four groups (no, mild, moderate and severe PH) with interpretable tricuspid and/or pulmonary regurgitation. ResultsNinety-seven dogs were included, with no (n = 29), mild (n = 13), moderate (n = 25) and severe (n = 30) PH. The intra- and inter-observer variability for EI measurements ranged from 2 % to 11 %. All EI were significantly elevated in severe compared to no, mild and moderate PH (P < 0.0005). In the moderate group, EIs and EIm were higher compared to the no PH group (P < 0.01). Tricuspid and pulmonary regurgitation pressure gradients and RHR parameters correlated with EId, EIs and EIm in all groups. Optimal cut-off values discriminating moderate and severe PH from no and mild PH were 1.24 (Sensitivity (Se) 60 %; Specificity (Sp) 90 %) for EId, 1.34 (Se 67 %; Sp 95 %) for EIs and 1.37 (Se 76 %; Sp 83 %) for EIm. ConclusionsLeft ventricular eccentricity indices are reproducible echocardiographic variables increasing with severity of PH. Dogs with moderate and severe PH can be discriminated from dogs with no or mild PH using EIs and EIm.

Noninvasive evaluation of pulmonary hypertension using the second heart sound parameters collected by a mobile cardiac acoustic monitoring system.

Pulmonary hypertension (PH) is linked to higher rates of morbidity and mortality worldwide. Early diagnosis of PH is important for clinical treatment. The estimated pulmonary artery systolic pressure (ePASP ≥ 35 mmHg) measured by echocardiography helps screen PH patients. In this paper, we report a novel PH screening method through a mobile cardiac acoustic monitoring system. In the retrospective study, patients admitted to our hospital between January 2022 and April 2023 were classified into PH and control groups using ePASP and compared with acoustic cardiographic parameters. According to ePASP, PH severity was classified as mild, moderate, and severe. We analyzed the first and second heart sound (S1, S2) characteristics, including amplitude (S1A, S2A), energy (S1E, S2E), and frequency (S1F, S2F). The study included 209 subjects, divided into PH (n = 121) and control (n = 88) groups. Pearson correlation analysis confirmed the positive correlation between S2F and ePASP. The diagnostic performance of S2F as assessed by the area under the ROC curve was 0.775 for PH. The sensitivity and specificity of diagnosing ePASP ≥ 35 mmHg when S2F ≥ 36 Hz were found to be 79.34% and 67.05%, respectively, according to ROC analysis. Severity classification was performed using S2F, the area under the ROC curve was 0.712-0.838 for mild PH, 0.774-0.888 for moderate PH, and 0.826-0.940 for severe PH. S2F collected by the mobile cardiac acoustic monitoring system offers a convenient method for remote PH screening, potentially improving PH management and outcomes.

Simultaneous bilateral pulmonary and cardiac invasion of metastatic testicular germ cell tumor: A rare case report

AbstractA 23‐year‐old man with a history of a testicular germ cell tumor (GCT) that was incompletely treated, is presented with dyspnea. Significant bilateral pulmonary masses were seen on computed tomography (CT). A large, hypermobile mass in the left atrium (LA) as well as mitral valve dysfunction and moderate pulmonary hypertension was detected by transthoracic echocardiography (TTE). Urgent cardiac surgery was performed to prevent the tumor embolism in addition to restore mitral valve function and reduce pulmonary hypertension. Pathologic reports confirmed testicular GCT. GCT metastasis to the heart and lungs is an incredibly rare phenomenon. To our knowledge, this is the first case of simultaneous GCT metastasis to both lungs and LA. This article highlights the importance of cardiac examinations and imaging in germ cell tumor patients. If there is a functional complication for vital organs such as the heart or lungs, surgical interventions are given priority before starting chemotherapy.

Qp/Qs in ostium secundum atrial septal defect mitigates right ventricular dysfunction in patients with secondary pulmonary hypertension

Abstract Background Ostium-secundum atrial septal defect (OS-ASD) can potentially lead to pulmonary hypertension (PH) and associated right ventricle (RV) - pulmonary artery (PA) uncoupling. This, in turn, may eventually result in RV systolic dysfunction. Notwithstanding, whether the characteristics of the OS-ASD could mitigate this relationship is unclear. Purpose To evaluate a potential interaction of the OS-ASD shunt (Qp/Qs ratio) on the relationship with RV–PA coupling and RV systolic function in patients with secondary PH. Methods We designed a cross-sectional study of 63 adult patients with OS-ASD in which we performed a conventional transthoracic echocardiogram using Vivid 9X-clear equipment (GE Vingmed Ultrasound, Horten, Norway). We evaluated the OS-ASD shunt and other echocardiographic parameters, including the tricuspid annular plane systolic excursion (TAPSE), the systolic pulmonary artery pressure (sPAP) ratio and the right ventricular free wall longitudinal strain (RVFWLS). Polynomic adjusted correlations were performed to model the relationship between the TAPSE/sPAP ratio and the RVFWLS, then we evaluated an interaction effect of the OS-ASD shunt (Qp/Qs ratio), respectively adjusted for age, sex, body mass index, body surface area and right atrial area. Results In our study, 49 (78%) subjects were male, with a mean age of 41.8 (±15) years; 7.94% (n=5) had mild PH, 42.9% (n=27) had moderate PH and 49.2% (n=31) had severe PH. Mean echocardiographic parameters were: TAPSE 24.5 (±5.1) mm , sPAP of 55.9 (±20.7) mmHg, TAPSE/sPAP 0.49 (±0.2) mm/mmHg, RVFWLS -25.6 (±6.6) % and Qp/Qs 2.9 (±0.9). We observed a quadratic relationship between the TAPSE/sPAP ratio and the RVFWLS (r=0.691, p &amp;lt; 0.001; Figure 1). OS-ASD shunt had a positive interaction effect (β=10.9, 95% CI: 2.09-19.6, p=0.018) on the relationship between TAPSE/sPAP and RVFWLS after adjusting for covariates (Figure 2). Conclusions In conclusion, in patients with OS-ASD, the RV systolic function decreases as the RV-PA uncoupling increases, as reflected in the quadratic regression between the TAPSE/sPAP and the RVFWLS. Moreover, the increase in OS-ASD shunt decreases RVFWLS values in relationship to the TAPSE/sPAP ratio as observed in the interaction model. These results could suggest that the OS-ASD characteristics could potentially mitigate RV dysfunction in patients with secondary PH.Figure 1.Quadratic regressionFigure 2.Interaction model

Differential Diagnosis of Cardiac Amyloidosis and Hypertrophic Cardiomyopathy

Diagnosis and differential diagnosis of cardiac amyloidosis and hypertrophic cardiomyopathy is difficult in some cases, which is confirmed by the presented clinical observation. The patient A., 67 years old, from the age of 59 for 7 years suffered from arterial hypertension with a maximum blood pressure of 170/100 mmHg, received hypotensive therapy. Myocardial infarction, a history of stroke denies. Since January 2018, at the age of 65, against the background of spontaneous stabilization of blood pressure figures, shortness of breath when climbing to the 2nd floor, lifting weights, suffocation at night, swelling of the shins, feet, in connection with which I turned to a doctor. When examined on an electrocardiogram, a low voltage of QRS complexes in the leads from the extremities was noted, there was no increase in the amplitude of the r wave in V1–3. Echocardiography revealed a thickening of the interventricular septum and the posterior wall of the left ventricle up to 1.9 cm without obstruction of the outlet of the left ventricle, restrictive type of diastolic dysfunction, dilation of the left and right atria, moderate pulmonary hypertension, moderate amount of fluid in the pericardial cavity. Magnetic resonance imaging of the heart revealed a pattern typical of cardiac amyloidosis: diffuse subendocardial contrast of the myocardium of both ventricles in the absence of local contractility disorders, increased myocardial thickness in all segments, hydropericardium. Biopsy of the skin and subcutaneous fat with Congo red staining and polarization microscopy revealed no amyloid deposits. No mutations in the transthyretin gene responsible for transthyretin amyloidosis (ATTR–amyloidosis) were detected during the genetic study. Sequencing of 10 genes encoding myocardial sarcomeric proteins in the MYBPC3 gene revealed a mutation c.3197C &gt;G (p.Pro1066Arg) in a heterozygous state, previously described in patients with hypertrophic cardiomyopathy of Slavic origin. Cascade family screening for the mutation was not carried out due to the fact that the patient did not know the father, the mother died at the age of 75 from heart failure, the only son died from an accident six months before the patient’s treatment. On 15.02.2019, the patient suffered a circulatory arrest with successful resuscitation measures. For the purpose of secondary prevention of sudden cardiac death, a single–chamber cardioverter-defibrillator was implanted on 22.02.2019. Despite the ongoing therapy, the patient died in March 2019. from progressive heart failure. Thus, a clinical case is presented where magnetic resonance imaging suspected amyloid cardiomyopathy, which did not receive morphological confirmation in biopsies of extra–cardiac localization. Hypertrophic cardiomyopathy caused by mutation c.3197C &gt;G (p.Pro1066Arg) in the MYBPC3 gene was confirmed on the basis of clinical and instrumental and molecular genetic methods. The pattern characteristic of cardiac amyloidosis described in this patient with instrumental examination methods may be due to a violation of autophagy processes previously described with a number of mutations in the MYBPC3 gene, which may lead to the accumulation of amyloid-like inclusions in cardiomyocytes. For differential diagnosis of cardiomyopathies in complex cases, endomyocardial biopsy may be required. The possibility of coexistence of genetically determined hypertrophic cardiomyopathy and amyloid heart disease is not excluded.

FRI506 Severe Dilated Cardiomyopathy As First Manifestation Of Thyrotoxicosis- A Case Report

Abstract Disclosure: Z. Saeed: None. C. Labib: None. W. Althunibat: None. M. Varatharajah: None. Introduction: Hyperthyroidism is a common metabolic disorder with prominent cardiovascular manifestations ranging from palpitations, angina-like chest pain, exercise intolerance and dyspnea to high-output heart failure and dilated cardiomyopathy. Approximately 6% of patients with hyperthyroidism/thyrotoxicosis develop symptoms of heart failure and less than 1% develop dilated cardiomyopathy with reduced left ventricular systolic function. Here we present a case of a 55-year-old man who presented with symptoms of heart failure and was diagnosed with dilated cardiomyopathy due to thyrotoxicosis. CASE PRESENTATION: 55-year-old man with a past medical history of vitiligo and cirrhosis presented to the emergency department with complaint of lower extremity swelling, cough, dyspnea on exertion, chest discomfort, palpitations and lethargy for two weeks. He also had a weight gain of 14 lbs. in this period. In the emergency department he had a heart rate of 118 beats/minute, physical exam revealed bilateral fine hand tremors, bibasilar crackles and a 2/6 holosystolic murmur on chest auscultation as well as hyperactive deep tendon reflexes. EKG showed atrial fibrillation with a rate of 184 beats/min. Initial chest imaging showed cardiomegaly, perihilar congestive changes and small right pleural effusion. CT abdomen &amp; pelvis demonstrated moderate volume ascites. Lab work showed a TSH of 0.00 mIU/L (0.5-5.0. mIU/L), T4 Free 5.3 ng/dL (0.9-2.3 ng/dL), Troponin T 36 ng/L, pro BNP 3150 pg/ml. He was given furosemide, propranolol, hydrocortisone, a bolus of diltiazem and was started on diltiazem and heparin drip and admitted to the hospital. Endocrinology was consulted and he was started on methimazole and methylprednisolone. Transthoracic echocardiogram showed an ejection fraction of 15%. Thyroid ultrasound was negative for any nodules or mass. Cardiac catheterization was done which revealed moderate pulmonary hypertension, non-ischemic dilated cardiomyopathy. Patient was discharged home on methimazole and guideline directed medical therapy for heart failure. He followed outpatient with Endocrinology and Cardiology. Six months after his hospital admission, repeat TSH was 1.71 mIU/L after treatment. Repeat echocardiography showed an improvement in ejection fraction from 15% to 35-40%. DISCUSSION: Prolonged untreated hyperthyroidism leads to high output heart failure characterized by an increase in heart rate, cardiac contractility and cardiac output which in some cases progresses to dilated cardiomyopathy with impaired left ventricular systolic function. Heart failure with reduced ejection fraction is a frequently encountered problem, most commonly due to ischemic cardiomyopathy. Thyrotoxicosis is one of the rare and reversible causes of nonischemic dilated cardiomyopathy which must be promptly diagnosed and treated in order to prevent life threatening complications. Presentation: Friday, June 16, 2023

Should All Patients With Pulmonary Hypertension Undergoing Non-Cardiac Surgery Be Managed by Cardiothoracic Fellowship-Trained Anesthesiologists?

To identify differences in practice patterns and outcomes related to the induction of general anesthesia for patients with pulmonary hypertension (PH) performed by anesthesiologists who have completed a cardiothoracic fellowship (CTA group) vs those who have not (non-CTA group). Retrospective study with propensity score matching. Operating room. All adult patients with PH undergoing general anesthesia requiring intubation at a single academic center over 5years. Patient baseline characteristics, peri-induction management variables, post-induction mean arterial pressure (MAP), and other outcomes were compared between CTA and non-CTA groups. Following propensity scoring matching, 402 patients were included in the final model, 100 in the CTA group and 302 in the non-CTA group. Also following matching, only cases of mild to moderate PH without right ventricular dysfunction remained in the analysis. Matched groups were overall statistically similar with respect to baseline characteristics; however, there was a greater incidence of higher ASA class (P = .025) and cardiology and thoracic procedures (P < .001) being managed by the CTA group. No statistical differences were identified in practice patterns or outcomes related to the induction of anesthesia between groups, except for longer hospital length of stay in the CTA group (P = .008). These results provide early evidence to suggest the induction of general anesthesia of patients with non-severe PH disease can be comparably managed by either anesthesiologists with or without a cardiothoracic fellowship. However, these findings should be confirmed in a prospective study.

Fenestration: Integrating Wings Into the Atrial Septal Occluder for Navigating a Challenging Terrain.

Background Atrial septal defect (ASD) closure with significant left-to-right shunt and concurrent comorbidities poses challenges for intervention. A fenestrated atrial septal defect (FASD) device is a viable option for patients who cannot undergo complete occlusion due to hemodynamic and medical reasons. This study explores the use of FASD occluders in patients with secundum ASD and associated comorbidities where complete occlusion is difficult. Methodology This retrospective study collected the details of patients recommended for FASD closure diagnosed with significant secundum ASD and who had additional comorbidities between July 2015 and July 2023 in a tertiary cardiac center in eastern India. Among this cohort, patients who underwent FASD device placement were subjected to a comprehensive analysis. Results In total, 16 patients diagnosed with secundum ASD, characterized by significant left-to-right shunt and concurrent comorbidities, were considered for FASD closure during the study period. Ultimately, 13 patients (first group) underwent fenestrated atrial septal occluder implantation. The average age was 45.07 years, with the majority being females (n = 9). Comorbidities among this cohort included substantial left ventricular diastolic dysfunction (n = 7), left ventricular diastolic dysfunction coupled with moderate pulmonary hypertension (n = 1), severe pulmonary hypertension (n = 1), severe pulmonary valvular stenosis with right ventricular diastolic dysfunction (n = 2), and systemic lupus erythematosus (SLE) (n = 2). From this cohort, three patients did not undergo the intervention. The second group consisted of an elderly patient with severe left ventricular diastolic dysfunction, a young adult with a history of left atrial arrhythmia, and achild with Duchenne muscular dystrophy (DMD). The average ASD size among patients who underwent the intervention was 26.38 mm, with a thick-to-thick dimension measuring 31.15 mm. The procedure was successful in all 13 patients, with the most frequently used device being a 34 mm occluder (range = 28-40 mm). All devices, excluding the initial one, were custom-made atrial septal occluders (Lifetech Scientific). Among the patients, 12 exhibited left-to-right fenestration flow, while one patient experienced fenestration constriction, likely due to occluder overcrowding. The first patient hada handmade 5 mm fenestration in a 40 mm Amplatzer septal occluder, which got closed off at the one-year follow-up. The procedure was well-tolerated hemodynamically in all patients, with no major complications during the peri-procedural period. Short-term follow-up indicated favorable patient progress. Conclusions FASD closure emerges as a pivotal alternative for intricate scenarios involving secundum ASD coupled with concurrent comorbidities, offeringindividualized tailored solutions. Alongside the conventional associated comorbidities, such as left ventricular diastolic dysfunction and pulmonary hypertension, FASD devices hold the potential to extend their benefits to patients grappling with other complexities, including severe pulmonary valvular stenosis, SLE, predisposition to left atrial arrhythmia, and conditions like DMD. Ensuring meticulous evaluation of patient suitability and providing ongoing vigilant care becomes paramount for achieving optimal outcomes. The validation of these findings and the broadening of the comprehension of this approach necessitate further comprehensive investigations.

Pulmonary Hypertension in Patients of Chronic Kidney Disease on Maintenance Hemodialysis: Study from a Tertiary Care Center in Central India

To study the incidence of pulmonary hypertension (PH) in chronic kidney disease (CKD) stage V patients on maintenance hemodialysis (HD) at our center. To compare clinical and metabolic variables among CKD patients with and without PH to search for possible etiologic factors. Comparison of PH in CKD patients at baseline and after 3 months of sildenafil therapy. The study was conducted in the Department of Nephrology, Sri Aurob-indo Institute of Medical Sciences, Indore, for a period of 1 year from December 2021 to November 2022. All CKD patients on maintenance HD at our center were included in the study. A pre-structured proforma was used to record patient data. Detailed clinical examination, 2DECHO, and Biochemical tests were done. All patients with mean pulmonary artery pressure (mPAP) &gt; 25 mmHg on 2D echocardiography were considered to have PH and were started on sildenafil therapy 20 mg three times a day for 3 months. PH was classified as mild PH (mPAP &gt; 25 up to 40 mmHg), moderate PH (mPAP &gt; 40 mmHg to 60 mmHg), and severe PH (mPAP &gt; 60 mmHg). Patients were then followed for 3 months to look for episodes of dyspnea and emergency admissions and reassessed after 3 months by repeat 2D echocardiography to find improvement in PH. A total of 102 patients were analyzed during the study period; among them, 40 patients (39.2%) had PH. Out of them, 18 patients (45%) had mild PH, 14 patients (35%) had moderate PH, and 8 patients (20%) patients had severe PH. Average age of our patients was 48.8 ± 9.4 years, the majority being men. On comparing the clinical features between patients with and without PH, none of the clinical parameters had any statistically significant impact on PH. Also, none of the laboratory parameters had statistical significance among PH and non-PH groups. Among the patients with PH, 25 patients (62.5%) had Arteriovenous (AV) fistula, 10 patients (25%) had temporary dialysis catheters. Eight patients (20%) had jugular catheters, two patients (5%) had femoral catheters, and 5 (12.5%) patients had tunneled jugular catheters. Initially, 102 patients were enrolled in the study. Of these, 40 (39.2%) had PH and 62 (60.7%) did not. Patients who had PH started sildenafil 20 mg three times a day. Of these 40 patients, at 3 months, eight patients were lost to follow-up, and 32 patients with PH remained in the study. Emergency admissions in each group of PH declined after 3 months, and the result was statistically significant. Echocardiographic findings were compared in patients with PH and without PH, but the difference in patients on HD with PH and without PH was not statistically significant. PH is a significant problem in CKD patients on HD. This issue needs to be evaluated in a timely manner to avoid the risk of morbidity and mortality. It is vital to treat them at the earliest to prevent life-threatening complications.

Case Report: Removal of septal occluder device and closure of atrial septal defect with pulmonary vein flow redirection

BACKGROUND: Atrial septal defect represents 10 to 15% of congenital heart disease and is more frequent in women with a 2:1 ratio. Sinus venosus atrial septal defect is usually associated with partial anomalous pulmonary venous drainage from the right superior pulmonary vein to the right atrium directly or indirectly through the superior vena cava. Rarer still, is the inferior sinus venosus, which may be associated with partial anomalous venous drainage from the right inferior pulmonary vein. CASE REPORT: 13-year-old patient with a history of intervention for interatrial defect closure with Amplatzer occluder at the age of six. He was brought to receive medical attention for fatigue and dyspnea that had been progressing for 2 years. Cardiac CT reported partial anomalous connection of pulmonary veins and echocardiogram reported a residual atrial septal defect of 10 mm and mild to moderate pulmonary hypertension. Based on the findings, surgical treatment was decided. EVOLUTION: In correction surgery, we observed Amplatzer occluder incorrectly positioned, the device was removed and flow was redirected from right pulmonary veins to left atrium with bovine pericardium patch. After the surgical intervention, the patient had a favorable evolution without no post-surgical complications. At follow-up, 15 days after medical discharge, the patient was asymptomatic, and a cardiac CT scan showed the corrected interatrial defect. CONCLUSION: The treatment of atrial septal defect should be individualized, since it will depend on the size of the defect, the age at the time of diagnosis, the magnitude of the shunt, the severity of the symptoms and the presence of other conditions. If the defect requires repair, it is important to weigh the benefits of choosing percutaneous closure vs. surgical intervention to avoid the need for reintervention, as in the present case. Closure and redirection of pulmonary vein flow with bovine patch is a safe and effective option.