Dilated Ventricle Research Articles

A Segmentation Framework with Unsupervised Learning-Based Label Mapper for the Ventricular Target of Intracranial Germ Cell Tumor.

Intracranial germ cell tumors are rare tumors that mainly affect children and adolescents. Radiotherapy is the cornerstone of interdisciplinary treatment methods. Radiation of the whole ventricle system and the local tumor can reduce the complications in the late stage of radiotherapy while ensuring the curative effect. However, manually delineating the ventricular system is labor-intensive and time-consuming for physicians. The diverse ventricle shape and the hydrocephalus-induced ventricle dilation increase the difficulty of automatic segmentation algorithms. Therefore, this study proposed a fully automatic segmentation framework. Firstly, we designed a novel unsupervised learning-based label mapper, which is used to handle the ventricle shape variations and obtain the preliminary segmentation result. Then, to boost the segmentation performance of the framework, we improved the region growth algorithm and combined the fully connected conditional random field to optimize the preliminary results from both regional and voxel scales. In the case of only one set of annotated data is required, the average time cost is 153.01s, and the average target segmentation accuracy can reach 84.69%. Furthermore, we verified the algorithm in practical clinical applications. The results demonstrate that our proposed method is beneficial for physicians to delineate radiotherapy targets, which is feasible and clinically practical, and may fill the gap of automatic delineation methods for the ventricular target of intracranial germ celltumors.

Measuring Single-Cell Calcium Dynamics Using a Myofilament-Localized Optical Biosensor in hiPSC-CMs Derived from DCM Patients.

Synchronized contractions of cardiomyocytes within the heart are tightly coupled to electrical stimulation known as excitation-contraction coupling. Calcium plays a key role in this process and dysregulated calcium handling can significantly impair cardiac function and lead to the development of cardiomyopathies and heart failure. Here, we describe a method and analytical technique to study myofilament-localized calcium signaling using the intensity-based fluorescent biosensor, RGECO-TnT. Dilated cardiomyopathy is a heart muscle disease that negatively impacts the heart's contractile function following dilatation of the left ventricle. We demonstrate how this biosensor can be used to characterize 2D hiPSC-CMs monolayers generated from a healthy control subject compared to two patients diagnosed with dilated cardiomyopathy. Lastly, we provide a step-by-step guide for single-cell data analysis and describe a custom Transient Analysis application, specifically designed to quantify features of calcium transients. All in all, we explain how this analytical approach can be applied to phenotype hiPSC-CM behaviours and stratify patient responses to identify perturbations in calcium signaling.

Cardiac abnormalities in stable chronic obstructive pulmonary disease: correlations and predictors

Abstract Background Patients with stable chronic obstructive pulmonary disease (COPD) are more likely to have cardiac abnormalities, which increase morbidity and mortality. The aim of the study is to explore correlations and probable predictors for these abnormalities in stable COPD patients. Patients and methods We conducted a prospective cohort study including 91 stable COPD cases, who were admitted to a tertiary health-care center during the period from August 2021 to January 2022. Spirometry, arterial blood gas, and ECG were performed for all participants. They were categorized using GOLD guidelines (2021) and assessed by two-dimensional Doppler echocardiography using the American and European Association of Echocardiography ASE recommendations. Results The study included 91 COPD patients; their mean age was 60.5 years with male predominance (62.6%). Of the patients, 76.9% were smokers and 49.5% of patients have respiratory failure type II. The most common abnormal ECG findings were P pulmonale, ventricular ectopic, and ischemic changes, which were more detected in severe COPD patients (94.29%). Echocardiographic assessment showed abnormal findings in almost 65%, more in the severe group. The most frequent findings were signs of pulmonary hypertension (PH) (72.5%), right ventricular dilatation (49.5%), and hypokinesia (21.98%). Risk factors of cardiac abnormalities were respiratory failure, COPD duration, comorbidities, and COPD stage. Conclusion Right ventricle dilatation, hypokinesia, and PH showed a positive correlation with echocardiographic findings and COPD severity. Respiratory failure and COPD duration were independent predictors of arrhythmia; COPD stage and the number of comorbidities were predictors of cardiac ischemia, while respiratory failure and comorbidities were predictors of PH.

New-onset atrial fibrillation in patients with SARS-CoV-2 pneumonia as a manifestation of acute myocardial injury

BACKGROUND: Over the past 3 years, the prevalence of atrial fibrillation (AF) has increased significantly worldwide, which was associated with the pandemic caused by SARS-CoV-2. It is accompanied by an increase in the cases of ischemic stroke, myocardial infarction, and development of heart failure due to acute myocardial injury. Given the high lethality of SARS-CoV-2 infection (COVID-19), studying the characteristics of new-onset AF is essential.
 AIM: The study aims at determining the predictors of new-onset AF in patients with COVID-19 pneumonia and at analyzing the clinical and pathophysiological characteristics of acute myocardial injury.
 MATERIALS AND METHODS: In 36 patients aged 4482 years (average 68.0) with COVID-19 pneumonia, AF paroxysms were recorded for the first time. All of them underwent computed tomography of the chest, electrocardiography, and echocardiography. The left ventricular ejection fraction was calculated using the Simpson method. Oxygen saturation was determined as blood oxygen saturation. Clinical blood tests were performed, C-reactive protein (CRP), ferritin, D-dimer, fibrinogen, and troponin I levels were measured.
 RESULTS: Along with the well-known predictors of AF development (arterial hypertension, coronary heart disease, left ventricular myocardial hypertrophy, and left atrial dilatation), with COVID-19 pneumonia, new-onset AF paroxysms were recorded in patients of the middle, elderly, and late-life age. In 44.4% of patients with AF, cardiomegaly occurred with dilatation of both atria and ventricles. With decreased left ventricular ejection fraction, the incidence of AF paroxysms reached 61.5%. With preserved ejection fraction, AF paroxysms occurred much less frequently (27%). In patients with AF, the extent of lung damage is on average 62.5% (2080%) with oxygen support saturation of 93% (7697%). Serum troponin I levels of 2000 ng/L indicated acute myocardial injury. CRP and blood ferritin values confirmed the presence of a pronounced inflammatory component in myocardial injury. High concentrations of blood fibrinogen and D-dimer, reaching 16,301 ng/mL, were associated with a tendency to hypercoagulation in patients with AF and COVID-19 pneumonia.
 CONCLUSIONS: COVID-19 has a direct damaging effect on the myocardium and probably persists for a long time, which may induce AF in patients with acute pneumonia.

Risk factors, treatment, and outcome in dogs and cats with subdural hematoma and hemispheric collapse after ventriculoperitoneal shunting of congenital internal hydrocephalus.

Overshunting and hemispheric collapse are well-known complications after ventriculoperitoneal shunt (VPS) implantation. Risk factors that predispose to overshunting, treatment options, and prognosis after therapeutic intervention have not been described. To identify preoperative risk factors for overshunting, the effect of surgical decompression, and their outcomes. Seventy-five dogs and 7 cats. Retrospective case cohort study. Age, breed, sex, body weight, number of dilated ventricles, ventricle brain ratio, intraventricular pressure, and implanted pressure valve systems were evaluated as possible risk factors. Overshunting had a prevalence of 18% (Cl 95% 9.9-26.66). An increase of 0.05 in VBR increased the risk of overshunting by OR 2.23 (Cl 95% 1.4-3.5; P = .001). Biventricular hydrocephalus had the highest risk for overshunting compared to a tri- (OR 2.48 with Cl 95% 0.5-11.1) or tetraventricular hydrocephalus (OR 11.6 with Cl 95% 1.7-81.1; P = .05). There was no influence regarding the use of gravitational vs differential pressure valves (P > .78). Overshunting resulted in hemispheric collapse, subdural hemorrhage, and peracute deterioration of neurological status in 15 animals. Subdural hematoma was removed in 8 dogs and 2 cats with prompt postoperative improvement of clinical signs. Biventricular hydrocephalus and increased VBR indicate a higher risk for overshunting. The use of differential valves with gravitational units has no influence on occurrence of overshunting related complications and outcomes. Decompressive surgery provides a favorable treatment option for hemispheric collapse and has a good outcome.

Right ventricular papillary muscle approximation to the septum as an adjunct technique during tricuspid valve repair in congenital heart surgery

ObjectiveTricuspid regurgitation is often caused by leaflet splaying from displaced papillary muscles or ventricular dilatation. Traditional annuloplasty may not address this mechanism. The present study describes a single institution's experience using right ventricular papillary muscle approximation for tricuspid valve repair. MethodsRight ventricular papillary muscle approximation consists of suturing the anterior papillary muscle to a point of the septum (septum or septal papillary muscle) that optimizes leaflet coaptation. We describe our technique and analyze clinical data of patients undergoing tricuspid valve repair with right ventricular papillary muscle approximation during congenital heart surgery between 2012 and 2021. ResultsRight ventricular papillary muscle approximation was performed as an adjunct procedure in 207 of 825 tricuspid valve repairs (25.1%). Discharge tricuspid regurgitation grade was mild tricuspid regurgitation or less in 153 patients (73.9%), and 140 patients (67.6%) had mild tricuspid regurgitation or less at a median latest follow-up of 3.2 years (interquartile range, 0.7-6.8). Thirty patients (14.5%) underwent 11 early tricuspid valve reinterventions (3 due to right ventricular papillary muscle approximation dehiscence) and 21 late tricuspid valve reinterventions. Estimated 5-year freedom from tricuspid valve reintervention was 84% (95% CI, 77.0-89.2). Systemic right ventricle physiology (odds ratio, 2.88, P = .048) and multiple mechanisms of tricuspid regurgitation (odds ratio, 7.35, P = .038) were significant predictors of tricuspid valve reintervention on multivariable analysis. ConclusionsTricuspid valve repair with right ventricular papillary muscle approximation demonstrates acceptable short-term durability, but similar to other tricuspid valve repair strategies is less durable in patients with systemic right ventricle pressure and multiple mechanisms of tricuspid regurgitation. Right ventricular papillary muscle approximation is a safe and effective adjunct technique that should be considered in patients with tricuspid regurgitation caused by leaflet splaying from displaced papillary muscles or right ventricle dilatation.

Acute Cor Pulmonale

In physiological conditions the left heart ventricle produces a higher pressure which aims to pump blood throughout the body while the right heart ventricle has a lower pressure to pump blood to the lungs. If pulmonary hypertension or increased blood pressure in the pulmonary arteries occurs, it can cause the right ventricle of the heart to work harder to pump blood against the high pressure. If there is pulmonary hypertension in the long term so that the right ventricle of the heart is burdened, it will cause cor pulmonale. Cor pulmonale is hypertrophy or dilatation of the right ventricle caused by pulmonary hypertension. Pulmonary hypertension is caused by diseases that attack the structure, function of the lungs or pulmonary blood vessels that can progress to right heart failure. Cor pulmonale can be acute or chronic. This literature review in made to know the theoretical concept of patients with cor pulmonale disorders including definitions, epidemiology, pathophysiology, clinical features, interventions, and prognosis and complications in acute cor pulmonale patients.

Alveolar and interstitial disease in chronic smoker presenting as combined pulmonary fibrosis with emphysema: Double trouble!

Abstract Tobacco smoke exposure causes diverse pulmonary manifestations with involvement of alveolar, interstitial, and vascular diseases due to inflammatory pathology apart from more lethal lung cancer. Combined pulmonary fibrosis and emphysema is a heterogenous lung disease documented in smokers, which includes emphysema in the upper lobes and pulmonary fibrosis in the lower lobes. In this case report, we have reported an 80-year-old male presented with progressive shortness of breath with fatigability and hypoxia treated as emphysema with inhaled bronchodilators. Response to medical treatment was not satisfactory with worsening of shortness of breath and fatigability. Clinical examination revealed that bilateral basal Velcro crepitation with resting oxygen saturation was 88% at room air. High-resolution computerized imaging documented emphysema in the upper lobes with honeycombing and the lower lobes with tractional bronchiectasis. Echocardiography documented pulmonary hypertension with dilated right atrium and ventricle. We have treated with oxygen supplementation during rest and ambulation, long-acting inhaled bronchodilator medicines, and antifibrotic nintedanib with strict counseling for the avoidance of tobacco exposure. Cardiopulmonary parameters’ improvement including in 6-min walk distance was significant with bronchodilators and antifibrotics.

Midline suprapineal pseudocyst in brain of fetuses with open spina bifida.

Recently, it was noted that fetuses with open spina bifida (OSB) may have a midline cystic structure evident on ultrasound. Our aims were to determine the prevalence of this cystic structure, shed light on its pathophysiology and investigate the association between its presence and other characteristic brain findings in fetuses with OSB. This was a single-center retrospective study of all fetuses with OSB and available cineloop images in the axial plane referred to the Ontario Fetal Centre, Toronto, Canada, between June 2017 and May 2022. Ultrasound and magnetic resonance imaging (MRI) data obtained between 18 + 0 and 25 + 6 weeks were reviewed in search of a midline cystic structure. Pregnancy and lesion characteristics were collected. Transcerebellar diameter (TCD), clivus-supraocciput angle (CSA) and additional brain abnormalities (abnormal cavum septi pellucidi (CSP), abnormal corpus callosum (CC) and periventricular nodular heterotopia (PNH)) were assessed. In cases of in-utero repair, imaging findings were reviewed postoperatively. In cases of termination, neuropathological findings were reviewed, if available. Of 76 fetuses with OSB, 56 (73.7%) had a suprapineal cystic structure on ultrasound. The percentage of agreement between ultrasound and MRI detection was 91.5% (Cohen's kappa coefficient, 0.78 (95% CI, 0.57-0.98)). Brain autopsy in terminated cases revealed a dilatation of the posterior third ventricle, with redundant tela choroidea and arachnoid forming the membranous roof of the third ventricle, anterior and superior to the pineal gland. A cyst wall could not be identified, indicating that the structure was a pseudocyst. The presence of the pseudocyst was associated with a smaller CSA (pseudocyst absent, 62.11 ± 9.60° vs pseudocyst present, 52.71 ± 8.22°; P = 0.04). When the pseudocyst was present, its area was correlated inversely with TCD (r, -0.28 (95% CI, -0.51 to -0.02); P = 0.04). Fetal surgery did not have any impact on the growth rate of the pseudocyst (fetal surgery, 5.07 ± 3.29 mm2 /week vs expectant management, 4.35 ± 3.17 mm2 /week; P = 0.58). The presence of the pseudocyst was not associated with abnormal CSP, CC or presence of PNH. None of the cases with available postnatal follow-up required surgical procedure related to the pseudocyst. Approximately 75% of all OSB cases have a suprapineal pseudocyst. Its presence is associated with the degree of hindbrain herniation but not with abnormalities of the CSP and CC or presence of PNH. Thus, it should not be regarded as additional brain pathology and should not preclude fetuses from undergoing fetal surgery for OSB. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

A paintable and adhesive hydrogel cardiac patch with sustained release of ANGPTL4 for infarcted heart repair

The infarcted heart undergoes irreversible pathological remodeling after reperfusion involving left ventricle dilation and excessive inflammatory reactions in the infarcted heart, frequently leading to fatal functional damage. Extensive attempts have been made to attenuate pathological remodeling in infarcted hearts using cardiac patches and anti-inflammatory drug delivery. In this study, we developed a paintable and adhesive hydrogel patch using dextran-aldehyde (dex-ald) and gelatin, incorporating the anti-inflammatory protein, ANGPTL4, into the hydrogel for sustained release directly to the infarcted heart to alleviate inflammation. We optimized the material composition, including polymer concentration and molecular weight, to achieve a paintable, adhesive hydrogel using 10% gelatin and 5% dex-ald, which displayed in-situ gel formation within 135 s, cardiac tissue-like modulus (40.5 kPa), suitable tissue adhesiveness (4.3 kPa), and excellent mechanical stability. ANGPTL4 was continuously released from the gelatin/dex-ald hydrogel without substantial burst release. The gelatin/dex-ald hydrogel could be conveniently painted onto the beating heart and degraded in vivo. Moreover, in vivo studies using animal models of acute myocardial infarction revealed that our hydrogel cardiac patch containing ANGPTL4 significantly improved heart tissue repair, evaluated by echocardiography and histological evaluation. The heart tissues treated with ANGPTL4-loaded hydrogel patches exhibited increased vascularization, reduced inflammatory macrophages, and structural maturation of cardiac cells. Our novel hydrogel system, which allows for facile paintability, appropriate tissue adhesiveness, and sustained release of anti-inflammatory drugs, will serve as an effective platform for the repair of various tissues, including heart, muscle, and cartilage.

Abstract P2135: A Novel Pkp2 Mouse Model Of Genetic Arrhythmogenic Right Ventricular Cardiomyopathy And Its Rescue By Gene Therapy

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disorder in which cardiomyocytes lack structural integrity and signaling is compromised resulting in the disruption of normal rhythms and formation of fibrosis. Patients suffer from arrhythmia and are at increased risk of sudden cardiac death and heart failure. Previous work has demonstrated that heterozygous mutations in the desmosomal gene, plakophilin-2 ( PKP2 ), are the most common cause of ARVC. Our objective was to investigate the mechanisms of ARVC development and test a potential ARVC gene therapy strategy using a novel mouse model of Pkp2. Methods: A knock-in (KI) mouse model with a point mutation corresponding to human PKP2 ( PKP2 c.2146-1G>C or Pkp2 KI) was generated via CRISPR/Cas9 technology. High fat diet (HFD) was used to induce the ARVC phenotype in the Pkp2 KI heterozygous (het) mice. The Pkp2 KI het mice were crossed with a cardiac-specific Pkp2 cKO (αMHC-Cre ERT ) mouse to generate a tamoxifen inducible model (KIxKO). Cardiac function was assessed by echocardiography and arrhythmia burden by EKG. The KIxKO animals received AAV:PKP2 via retro-orbital injection. Results: Pkp2 KI het animals with normal chow had slow and mild ARVC development, and 50% decrease in PKP2 protein expression, with right ventricle (RV) dilation not occurring until animals were 9 months old. Induction with HFD from 8 weeks hastened and worsened the RV dilation. In contrast, the KIxKO mice exhibited >90% decrease in PKP2 protein expression and a correspondingly severe ARVC phenotype starting from 3 weeks after Tamoxifen induction. This included a high arrhythmia burden and both RV area dilation and decreased ejection fraction, indicating the mice eventually progressed to biventricular heart failure. This resulted in 100% mortality in KIxKO animals by 9 weeks post-tamoxifen induction. A single dose of AAV:PKP2 gene therapy (6E13 vg/kg) maintained ejection fraction, prevented ARVC development and improved survival in the KIxKO mice after 9 weeks of treatment. Conclusion: The current results demonstrated that AAV:PKP2 gene therapy may be a promising therapeutic approach to treat ARVC patients with PKP2 mutations resulting in haploinsufficiency.

Prognostic value of right ventricular dilatation on computed tomography pulmonary angiogram for predicting adverse clinical events in severe COVID-19 pneumonia.

Right ventricle dilatation (RVD) is a common complication of non-intubated COVID-19 pneumonia caused by pro-thrombotic pneumonitis, intra-pulmonary shunting, and pulmonary vascular dysfunction. In several pulmonary diseases, RVD is routinely measured on computed tomography pulmonary angiogram (CTPA) by the right ventricle-to-left ventricle (LV) diameter ratio > 1 for predicting adverse events. The aim of the study was to evaluate the association between RVD and the occurrence of adverse events in a cohort of critically ill non-intubated COVID-19 patients. Between February 2020 and February 2022, non-intubated patients admitted to the Amiens University Hospital intensive care unit for COVID-19 pneumonia with CTPA performed within 48 h of admission were included. RVD was defined by an RV/LV diameter ratio greater than one measured on CTPA. The primary outcome was the occurrence of an adverse event (renal replacement therapy, extracorporeal membrane oxygenation, 30-day mortality after ICU admission). Among 181 patients, 62% (n = 112/181) presented RVD. The RV/LV ratio was 1.10 [1.05-1.18] in the RVD group and 0.88 [0.84-0.96] in the non-RVD group (p = 0.001). Adverse clinical events were 30% and identical in the two groups (p = 0.73). In Receiving operative curves (ROC) analysis, the RV/LV ratio measurement failed to identify patients with adverse events. On multivariable Cox analysis, RVD was not associated with adverse events to the contrary to chest tomography severity score > 10 (hazards ratio = 1.70, 95% CI [1.03-2.94]; p = 0.04) and cardiovascular component (> 2) of the SOFA score (HR = 2.93, 95% CI [1.44-5.95], p = 0.003). Right ventricle (RV) dilatation assessed by RV/LV ratio was a common CTPA finding in non-intubated critical patients with COVID-19 pneumonia and was not associated with the occurrence of clinical adverse events.

Effect of Preoperative Volume Overload on Left Ventricular Function Recovery After Ventricular Septal Defect Repair

Significant left-to-right shunt across a ventricular septal defect (VSD) may lead to left ventricle (LV) volume overload and dilation. The acute loss of LV preload after repair of VSD may contribute to postoperative LV systolic dysfunction. The primary aim of the study is to assess the effect of presence of preoperative LV dilation on LV systolic function recovery after VSD repair. We evaluated the LV systolic function by measuring LV longitudinal strain and ejection fraction (EF) before surgery (time point 1) and at 5 time points after VSD repair (time point 2: 0 to 2 weeks, time point 3: 2 to 6 weeks, time point 4: 6 weeks to 4 months, time point 5: 4 to 12 months, and time point 6: >12 months). A total of 120 patients were included in the study cohort. A total of 84 patients (70%) had LV dilation (group 1) and 36 patients (30%) had normal LV size on preoperative echocardiogram (group 2). Median age (interquartile range 25% to 75%) at surgery was 5.5 months (4 to 10) and 7 months (5 to 44.5) in groups 1 and 2 respectively (p=0.03). Mean LV EF and strain were not significantly different among the 2 groups at time point 1. At time point 2, both mean EF and strain were significantly lower in group 1 compared with group 2 (p <0.05). At time point 3, mean EF was not significantly different among the two groups, while mean LV strain was significantly lower in group 1 (p=0.044). At time points 4, 5 and 6, mean EF and strain were not significantly different between the two groups. In conclusion, presence of preoperative LV dilation is associated with a more pronounced LV systolic dysfunction in the early postoperative period only. The LV systolic function recovers back to the baseline after the first year following the repair. These are very reassuring prognostic findings.

Endoscopic Excision of Third Ventricle Choroid Plexus Papilloma

Abstract Introduction Choroid plexus papilloma is a rare intraventricular tumor in children. Its management poses a challenge. Here we describe such a case of third ventricular choroid plexus papilloma and its endoscopic excision through a single burr hole. Case Description A 1-year-old child presented with headache, vomiting, and a large head with sunset sign and tense fontanel. Computed tomography (CT) and magnetic resonance imaging brain plain and contrast revealed dilated ventricles with a frondlike mass in the third ventricle with intense contrast enhancement, suggestive of choroid plexus papilloma of the third ventricle. The lesion was excised completely using an endoscope placed through a single burr hole at the right Kocher's point. Postoperative recovery was uneventful. The child was relieved of his symptoms. CT scan revealed complete removal of the lesion. Histopathology confirmed the diagnosis of choroid plexus papilloma. Results and Conclusion We document our surgical experience and present an edited video of the surgery. The key steps and nuances are described in the audio timeline. The authors acknowledge the feasibility of performing this complex surgery via a minimally invasive method, which has not been accepted routinely for this pathology.

Clinical and Paraclinical Findings of Primary Pulmonary Hypertension in Children

Background: Given the significant morbidity and mortality of juvenile pulmonary arterial hypertension (PAH), understanding the clinical and paraclinical findings of this condition can aid in the early diagnosis and identification of helpful factors in its treatment. Objectives: This study aimed to evaluate the clinical and paraclinical findings of idiopathic pulmonary arterial hypertension (iPAH) in children. Methods: This cross-sectional study was carried out on 20 children with iPAH who were assessed in terms of pulmonary hypertension or hospitalized at the cardiac ward of Imam Reza hospital, Mashhad, Iran, during January 2018 to October 2001. The clinical data, chest X-ray (CXR), electrocardiographic, echocardiographic, cardiac catheterization findings, and mortality results were extracted from the medical records and recorded in a researcher-made questionnaire. Results: The current study’s findings revealed a higher prevalence of iPAH in females (70%) than men, as well as in term neonates compared to preterm newborns. The most common clinical findings were active dyspnea, heart murmur, and cyanosis. Prominent pulmonary conus, right atrial enlargement (RAE), and right ventricular hypertrophy (RVH) were the most common CXR findings. The right axis deviation, RVH, and RAE were the most common abnormal findings of electrocardiography, respectively. Based on echocardiographic findings, dilated right atrium and dilated right ventricle were reported in all subjects. The mean of tricuspid regurgitation pressure gradient (TRPG) and peak systolic pressure gradient (PSPG) were 76.33 ± 22.8 and 44.2 ± 13.18 mmHg, respectively. The mean ratios of systolic pulmonary artery pressure (PAP), diastolic PAP, and mean pulmonary artery pressure (mPAP) to aortic systolic pressure were estimated at 0.92, 0.78, and 0.88, respectively. The results confirmed a significant relationship between mPAP and consanguinity (P = 0.03). In addition, the findings demonstrated a correlation between echocardiographic findings (i.e., TRPG and PSPG) and systolic PAP, diastolic PAP, and mPAP (P &lt; 0.05). In total, 56.3% of the patients expired, most of whom (37.5%) were the younger children. Conclusions: The findings suggest that IPAH should be considered as a possible diagnosis in various clinical manifestations, including respiratory distress, hepatomegaly, ascites and even neurological symptoms. Since there are no specific symptoms, therefore a set of history, physical examination, laboratory findings, and clinical suspicion should be taken into consideration.

Pitfalls in the Diagnosis of Peri-Operative Acute Pulmonary Embolism: A Case Report

An 80 years-old man with hypertension, atrial fibrillation, and coronary artery occlusion disease presented for an elective right femur bipolar hip arthroplasty under general anesthesia. Preoperative transthoracic echocardiography (TTE) showed only preexisting regional wall motion abnormality. During the reversal of muscular relaxation following surgery, there was a sudden decrease in blood pressure associated with rapid ventricular response, which was successfully treated by cardioversion. No decrease in end-tidal carbon dioxide level was observed during the hypotensive episode. However, despite fluid and blood resuscitation and vasopressor treatment, there was a repeated hemodynamic collapse postoperatively. A portable TTE showed dilated right atrium and right ventricle (RV), D-shaped left ventricle, and septal wall motion abnormalities consistent with RV pressure overload (McConnell’s sign). Computed tomography pulmonary angiography revealed multifocal PE (pulmonary embolism). Anticoagulation therapy was continued to avoid PE recurrence. The patient discharged uneventfully 30 days later. Perioperative PE is a rare and perhaps dangerous circumstance that can be challenging to diagnose when it happens under anesthesia. The likelihood of PE should always be suspected at the time of perioperative hemodynamic collapse.

Improvement of left ventricular ejection fraction in patients with heart failure with reduced ejection fraction: Predictors and clinical impact

BackgroundA percentage of patients with heart failure with reduced ejection fraction (HFrEF) improve left ventricular ejection fraction (LVEF) in the evolution. This entity, defined for the first time in an international consensus as heart failure with improved ejection fraction (HFimpEF), could have a different clinical profile and prognosis than HFrEF. Our main aim was to analyze the differential clinical profile between the two entities, as well as the mid-term prognosis. Material-methodsProspective study of a cohort of patients with HFrEF who had echocardiographic data at baseline and follow-up. A comparative analysis of patients who improved LVEF with those who did not was made. Clinical, echocardiographic and therapeutic variables were analyzed, and the mid-term impact in terms of mortality and hospital readmissions for HF was assessed. ResultsNinety patients were analyzed. Mean age was 66.5(10.4) years, with a male predominance (72.2%). Forty five patients (50%) improved LVEF (Group-1,HFimpEF) and forty five patients (50%) sustained reduced LVEF (Group-2,HFsrEF). The mean time to LVEF improvement in Group-1 was 12.6(5.7) months. Group-1 had a more favorable clinical profile: lower prevalence of cardiovascular risk factors, higher prevalence of de novo HF (75.6% vs. 42.2%; p<0.05), lower prevalence of ischemic etiology (22.2% vs. 42.2%; p<0.05), with less basal dilatation of the left ventricle. At the end of follow-up (mean 19(1) months) Group-1 had a lower hospital readmission rate (3.1% vs. 26.7%; p<0.01), as well as lower mortality (0% vs. 24.4%; p<0.01). ConclusionPatients with HFimpEF seem to have a better mid-term prognosis in terms of reduced mortality and hospital admissions. This improvement could be conditioned by the clinical profile of patients HFimpEF.

Combined pulmonary fibrosis with emphysema in nonsmokers needs connective tissue disease workup: A case report

Combined pulmonary fibrosis and emphysema (CPFE) is a heterogenous lung disease documented in smokers, which includes emphysema in upper lobes and pulmonary fibrosis in lower lobes. CPFE in nonsmokers is rare, and very few literatures are available. In this case report, we have reported an 80-year-old male presented with progressive shortness of breath with fatigability and hypoxia treated as emphysema with inhaled bronchodilators. Response to medical treatment was not satisfactory with worsening of shortness of breath and fatigability. Clinical examination revealed bilateral basal velcro crepitations with resting oxygen saturation was 88% at room air. High-resolution computerized imaging documented emphysema in upper lobes with honeycombing and tractional bronchiectasis in lower lobes. Echocardiography documented pulmonary hypertension with dilated right atrium and ventricle. Connective tissue disease (CTD) panel documented workup positive for polymyositis. We have treated with oxygen supplementation during rest and ambulation, long-acting inhaled bronchodilator medicines, antifibrotic nintedanib, anti-inflammatory methylprednisolone, and pulmonary vasodilator tadalafil. Improvement in cardiopulmonary parameters including in 6-minute walk distance was significant with bronchodilators, mycophenolate mofetil, tadalafil, and antifibrotics.

Cardiac dysfunction and final outcome in correlation with serum cortisol level in active pulmonary tuberculosis: A single-center experience

Introduction: Although cardiac involvement is extremely rare in tuberculosis (TB), cardiac dysfunction is not uncommon. We have studied the prevalence of cardiac dysfunction in pulmonary TB with special emphasis on echocardiography, serum cortisol, and its correlation in cases with unstable cardiorespiratory parameters. Methods: A prospective observational and interventional study conducted in Pulmonary Medicine, MIMSR Medical College and Venkatesh Chest Hospital, Latur, India during July 2015 to December 2020, which included 800 cases of active pulmonary TB confirmed microscopically or with GeneXpert MTB/RIF, documented MTB genome in respiratory specimens’ sputum/induced sputum and bronchoscopy-guided bronchial wash, or bronchoalveolar lavage whenever necessary. Cases with known risk factors for cardiac disease and taking cardiac medicines and cases with pericardial effusion were excluded from study. Disproportionate tachycardia and tachypnea with or without shock and hypoxia were the key entry point criteria in this study. Chest radiograph, pulse oximetry, electrocardiography (ECG), sputum examination, cardiac enzyme studies (creatine phosphokinase MB [CPK-MB] and cardiac troponins), serum cortisol test, and echocardiography are done in all study cases during enrollment at 2 and 6 months of treatment with anti-TB medicines. Statistical analysis was carried out by chi-square test. Observations and analysis: Of the 800 cases with active pulmonary TB, cardiac dysfunction was documented in 26% cases, 44% were females, and 56% cases were older than 50 years. Echocardiographic abnormalities were documented such as global hypokinesia in 62% cases; depressed left ventricular systolic and diastolic function in 44 and 28% cases, respectively; dilated right atrium and right ventricle in 32% cases; and pulmonary hypertension in 6% cases. Serum cortisol level was significantly lower in cases with cardiac dysfunction (P &lt; 0.00001). Hypoxia had significant association with right and left heart dysfunction (P &lt; 0.00001). Cachexia, anemia, and hypoalbuminemia were documented to have significant association with cardiac dysfunction (P &lt; 0.00001). Treatment outcome showed significant improvement in cardiac function (P &lt; 0.00001). Coronary angiography did not show significant coronary artery lesions, and computed tomography (CT) pulmonary angiography did not show pulmonary embolism. Conclusion: Cardiac dysfunction in active pulmonary TB is underestimated and less evaluated routinely; disproportionate tachycardia and tachypnea with or without shock are clinical indicators to suspect early, especially in cases with risk factors such as advanced pulmonary TB on chest radiograph, cachexia with body mass index (BMI) &lt;18, advanced age, females, cases with anemia, and hypoalbuminemia. Echocardiography shows “global hypokinesia” as a predominant cardiac dysfunction in study cases, and right or left heart dysfunction depends on with or without hypoxia, respectively. Serum cortisol measurement will help in majority of the cases and a proportionate number of cases were having associated adrenal suppression.

Experience of Surgical Treatment of Complex Forms of Ebstein’s Anomaly through the Method of Hemodynamic Correction

Ebstein’s anomaly (EA) is a rare and complex congenital heart disease, with a prevalence of 4 to 7 cases per 100,000 live births. At this stage of the development of EA surgery, significant progress has been made in our understanding and treatment of EA, but the question of the tactics of treating complex forms, especially in the period of infancy and early childhood, remains open. Due to significant phenotypic heterogeneity, the treatment of EA varies greatly from patient to patient and depends on many factors that require a comprehensive approach. The choice of the method of surgical correction directly depends on the anatomical features: the degree of dysplasia, rotation and displacement of the tricuspid valve, the degree of atrialization of the right ventricle, the combination with other congenital heart diseases, and the severity of hemodynamic disorders.&#x0D; The aim. To evaluate perioperative characteristics in patients with complex forms of EA, immediate and long-term results after hemodynamic correction.&#x0D; Materials and methods. In the period from 1996 to 2022, at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine, one and a half ventricle repair was performed in 18 patients with complex forms of EA. There were 12 (67%) male patients, 6 (33%) female patients. The median age of the patients at the time of surgery was 30 months [3.5; 312]. The main diagnostic method in determining the defect and assessing the immediate and remote results was echocardiography and probing of the heart cavities. In order to qualitatively evaluate the perioperative, immediate and long-term results to determine the optimal tactics and terms of surgical intervention, prevention of possible complications, all the patients were divided into two age groups: I group up to 2.5 years including 9 patients (50%), II group older than 2.5 years including 9 patients (50%).&#x0D; Results. In the postoperative period, 2 (11%) deaths were noted in both groups. In all the deceased patients, the cause of death was acute heart failure and acute respiratory failure. The analysis of the postoperative period showed normal results in such a cohort of highly complex patients of both groups, but a more stable course was noted among patients of the I group, as indicated by statistically significant values for the duration of mechanical ventilation, stay in the intensive care unit, stay in the hospital, the average dose of sympathomimetic support and its duration, the total duration of exudation in the comparison groups.&#x0D; An uncomplicated course of the early postoperative period was observed in 8 (44%) patients. The remaining 10 (56%) patients of both groups had: I group, 3 (33%) patients: 7 complications; II group, 5 (55%) patients: 13 complications. In 5 patients (2 patients in I group and 3 patients in II group), attention-grabbing acute heart failure was observed, which required high doses and long-term sympathomimetic support, and in 2 patients caused death. The average observation period was 45 months [6; 190]. No deaths were observed during the observation period among 13 (72%) patients of both groups, contact was lost with 3 (17%) patients. There were no violations in the functioning of the right ventricle, but the recovery of the systolic function of the left ventricle occurred only in 8 (44%) patients.&#x0D; Tricuspid insufficiency was minimal in 5 (28%) patients, moderate in 6 (33%), severe in 2 (11%) patients.&#x0D; Conclusions. Determining the tactics of treatment of complex forms of EA is a serious problem in patients of various ages, especially in newborns and infants, due to pronounced dilatation and systolic dysfunction of the left ventricle, a sharp decrease in systemic output due to changes in the geometry of the left ventricle. The technique of hemiventricular correction shows very promising results and may become the technique of choice for patients with severe forms of EA and pronounced right ventricular insufficiency.