High Pulmonary Vascular Resistance Research Articles

PULMONARY HYPERTENSION AND CONGENITAL HEART DISEASE: THE CLINIC BEYOND THE EVIDENCE

Abstract Eisenmenger syndrome (ES), first described by Victor Eisenmenger, was defined by Paul Wood in pathophysiologic terms as “pulmonary hypertension (PH) at systemic level, caused by a high pulmonary vascular resistance (PVR), with reversed or bidirectional shunt at aorto–pulmonary, ventricular, or atrial level.” It represents the most severe hemodynamic phenotype of pulmonary arterial hypertension (PAH) in association with congenital heart disease (CHD) and develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD. Although therapeutic management has rapidly advanced in recent years, these patients were not included in randomized controlled trials for specific pulmonary arterial hypertension drugs, except for bosentan. However, in clinical practice we apply treatment strategies combining drugs targeting multiple pathobiological pathways. The clinical case we describe is that of a 44–year–old patient, suffering from Down syndrome, with ES due to subaortic VSD and a a patency of Botallo duct, followed in our PH clinic of the "Ruggi D‘Aragona" University Hospital for about 9 years. The patient, on dual therapy with Macitentan 10 mg and Sildenafil 60 mg, was in the intermediate–high risk class, stationary. During the last two checks, there was a progressive worsening of the hemodynamic parameters, walking distance of only 100 meters, with SpO2 at baseline 90% and desaturation during the 6mwt. On cardiac color Doppler ultrasound evidence of TAPSE 18 mm, PAPS 100 mmHg, RAA 20 cm²; TAPSE/PAPS 0.18. The patient presented in WHO class 3. The only RCTs currently available concern the use of bosentan, but in clinical practice, initial monotherapy with an endothelin receptor antagonist or a phosphodiesterase type 5 inhibitor for symptomatic patients is proposed. Reassess and consider escalation to a double combination therapy in case of clinical worsening or inadequate clinical response. Consider uptitration with the addition of prostanoids when inadequate response and timely assess the eligibility for transplantation. For our patient therefore, in consideration of the impossibility of surgery, although there is little evidence on prospective observational studies based on cohorts of a few patients, we added selexipag 200 ug x 2/day to titrate therapy.This case opens the door to still unclear scenarios in which unfortunately the only weapon available remains the clinician‘s judgment and experience.

Prognostic performance of pulmonary effective arterial elastance in patients with heart failure

Objective: To explore the predictive value of pulmonary effective arterial elastance (Ea) in patients with heart failure (HF). Methods: This is a retrospective cohort study, which retrospectively included 284 patients with HF who underwent right heart catheterization at Heart Failure Center in Fuwai Hospital between September 2013 and February 2022. Data regarding baseline clinical characteristics, hemodynamic profiles, and prognosis were collected. Ea was calculated as mean pulmonary arterial pressure/stroke volume. Patients were divided into Ea<0.555 group and Ea≥0.555 group according to the median value of Ea (0.555 mmHg/ml, 1 mmHg=0.133 kPa). The primary outcome was the primary clinical event, set as the first occurrence of a series of composite events, including all-cause death, heart transplantation, left ventricular assist device implantation, and HF rehospitalization. Event-free survival was defined as the absence of primary clinical events. Spearman correlation analysis was used to calculate the correlation coefficient between Ea and parameters reflective of right heart function. The Kaplan-Meier analysis was used to compare the different groups for the estimation of outcomes with the log-rank test. We used Cox proportional-hazards regression models to estimate hazard ratios (HR) for primary clinical event. Subgroup analysis was performed based on the age, gender, New York Heart Association (NYHA) functional class, left ventricular ejection fraction, presence of pulmonary hypertension, and serum N-terminal pro-B-type natriuretic peptide (NT-proBNP) values. We used receiver operating characteristic (ROC) curve to calculate the area under the curve (AUC) of Ea for predicting event-free survival in patients with HF. Results: The median age was 51 years, and 206 (72.5%) patients were male. Ea and pulmonary vascular resistance (PVR) were significantly correlated (r=0.698, P<0.001). The correlation between Ea and pulmonary arterial elastance (PAC) were even more significant (r=-0.888, P<0.001). Compared with Ea<0.555 group, Ea≥0.555 group presented with higher serum NT-proBNP values (4 443 (1 792, 8 554) ng/L vs. 1 721 (480, 4 528)ng/L,P<0.001), higher PVR (3.4 (2.5, 4.7) Wood vs. 1.4 (0.9, 2.2) Wood, P<0.001), lower cardiac output (3.0 (2.3, 3.9) L/min vs. 4.3 (3.8, 4.9) L/min, P<0.001), and lower PAC (1.6 (1.3, 2.0) ml/mmHg vs. 4.0 (3.0, 6.0) ml/mmHg, P<0.001). The median follow-up time was 392 (166, 811) days. The Kaplan-Meier survival curve demonstrated a lower event-free survival rate in the Ea≥0.555 group compared to the Ea<0.555 group (Plog-rank<0.001). After multivariate adjustment, Ea (HR=1.734, P<0.001) remained significantly associated with the primary outcome. Subgroup analysis indicated that Ea was associated with the primary outcome across all subgroups. The AUC was 0.724 (P<0.001) for Ea to predict event-free survival calculated from ROC analysis. Conclusions: Ea is closely related to parameters reflective of right ventricular afterload. Increased Ea is an independent predictor of adverse outcomes in patients with HF.

Anesthesia management of laparoscopic right colectomy in an older patient with postoperative tetralogy of Fallot with residual anomaly

BackgroundDiversity in hemodynamics of adult congenital heart disease necessitates a case-by-case selection of appropriate surgical and anesthetic options. However, previous case reports regarding the management of laparoscopic surgery in adult patients with congenital heart disease are limited.Case presentationA 72-year-old man who underwent a laparoscopic right colectomy for colon cancer had a residual ventricular septal defect and right ventricular outflow tract obstruction despite post-repair of tetralogy of Fallot. Pulmonary hypertension or right ventricular dysfunction was not observed. The preoperative pulmonary to systemic blood flow ratio (Qp/Qs) was 2.3. After positive pressure ventilation and insufflation, the amount of left-to-right ventricular shunting decreased, and the Qp/Qs approached 1.0, as calculated from pulmonary arterial and systemic arterial blood gas analysis.ConclusionsLaparoscopic surgery might be tolerable in patients with tetralogy of Fallot who have preserved the right ventricular function, left-to-right ventricular shunting, and no high pulmonary vascular resistance.

(960) - High Preoperative Body Mass Index and Pulmonary Vascular Resistance Do Not Impact Survival After Pulmonary Thromboendarterectomy

(960) - High Preoperative Body Mass Index and Pulmonary Vascular Resistance Do Not Impact Survival After Pulmonary Thromboendarterectomy

Association of pulmonary hypertension with the outcome in patients undergoing edge-to-edge mitral valve repair

ObjectivesThe association of pulmonary hypertension (PH) with the outcome after mitral transcatheter edge-to-edge repair (M-TEER) focusing on the new ESC/ERS guidelines definition for PH.BackgroundPH is frequently found in patients with...

Effect of Maternal Preeclampsia on Cardiac Structure and Function in Very Low Birth Weight Infants.

We aimed to determine whether exposure to severe maternal preeclampsia (PE) in very low birth weight (VLBW) infants is associated with hypertrophic cardiac changes and altered hemodynamics. Case-control study of VLBW infants born at Los Angeles General Medical Center from May 2015 to August 2023, who had an echocardiogram within the first 7 days of life. Cases were infants exposed to maternal PE and controls were infants not exposed to maternal PE matched by birth weight (BW) 1:1. Laboratory, placental pathology results, hemodynamic data and clinical outcomes were collected and compared between cases and control infants. A total of 43 cases matched by BW with control infants were studied. There were no significant anatomical cardiac changes by echocardiography between cases and control infants. Cases had significantly higher blood pressure within the first 72 hours of life and lower ejection fraction (EF), fractional shortening, and peak systolic flow velocity through their patent ductus arteriosus (PDA) within the first week of life. Cases were more likely to be smaller despite being born at a later gestational age (GA), as well as small for GA with placental weight less than 10th percentile compared to control infants. Our findings indicate that infants born to mothers with PE have higher systemic vascular resistance as evidenced by elevated blood pressure, and lower EF and shortening fraction and higher pulmonary vascular resistance as evidenced by lower peak flow velocity through the PDA. We did not observe hypertrophic cardiac changes in exposed infants. These findings should be considered in clinical decision-making during management of these infants. · VLBW infants exposed to severe PE have higher rate of Small for gestational age and smaller placentas.. · VLBW infants exposed to severe PE have higher systemic vascular resistance during transitional period and lower EF and fractional shortening.. · VLBW infants exposed to severe PE have higher pulmonary vascular resistance..

Clinical phenogroup diversity and multiplicity: Impact on mechanisms of exercise intolerance in heart failure with preserved ejection fraction.

We aimed to clarify the extent to which cardiac and peripheral impairments to oxygen delivery and utilization contribute to exercise intolerance and risk for adverse events, and how this relates to diversity and multiplicity in pathophysiologic traits. Individuals with heart failure with preserved ejection fraction (HFpEF) and non-cardiac dyspnoea (controls) underwent invasive cardiopulmonary exercise testing and clinical follow-up. Haemodynamics and oxygen transport responses were compared. HFpEF patients were then categorized a priori into previously-proposed, non-exclusive descriptive clinical trait phenogroups, including cardiometabolic, pulmonary vascular disease, left atrial myopathy, and vascular stiffening phenogroups based on clinical and haemodynamic profiles to contrast pathophysiology and clinical risk. Overall, patients with HFpEF (n = 643) had impaired cardiac output reserve with exercise (2.3 vs. 2.8 L/min, p = 0.025) and greater reliance on peripheral oxygen extraction augmentation (4.5 vs. 3.8 ml/dl, p < 0.001) compared to dyspnoeic controls (n = 219). Most (94%) patients with HFpEF met criteria for at least one clinical phenogroup, and 67% fulfilled criteria for multiple overlapping phenogroups. There was greater impairment in peripheral limitations in the cardiometabolic group and greater cardiac output limitations and higher pulmonary vascular resistance during exertion in the other phenogroups. Increasing trait multiplicity within a given patient was associated with worse exercise haemodynamics, poorer exercise capacity, lower cardiac output reserve, and greater risk for heart failure hospitalization or death (hazard ratio 1.74, 95% confidence interval 1.08-2.79 for 0-1 vs. ≥2 phenogroup traits present). Though cardiac output response to exercise is limited in patients with HFpEF compared to those with non-cardiac dyspnoea, the relative contributions of cardiac and peripheral limitations vary with differing numbers and types of clinical phenotypic traits present. Patients fulfilling criteria for greater multiplicity and diversity of HFpEF phenogroup traits have poorer exercise capacity, worsening haemodynamic perturbations, and greater risk for adverse outcome.

Pulmonary vascular dysfunction without pulmonary hypertension: A distinct phenotype in idiopathic pulmonary fibrosis.

Pulmonary vascular dysfunction in the absence of pulmonary hypertension (PH) has been observed in patients with idiopathic pulmonary fibrosis (IPF). We describe the prevalence and etiology of elevated pulmonary vascular resistance (PVR) without PH among patients with IPF. Hemodynamic, echocardiographic, and functional respiratory imaging (FRI) data was compared between patients with IPF without PH with normal (<3 wood units) and elevated PVR (≥3 wood units). Mortality between these two groups were compared to patients with IPF and PH. Of 205 patients with IPF, there were 146 patients without PH, of whom 114 (78.1%) had a normal PVR and 32 (21.9%) who had a high PVR. Functional testing and hemodynamics were similar in the two groups, except for the cardiac index which was significantly lower in patients with a high PVR (2.3 vs. 2.6 L/min/m2; p = 0.004). Echocardiographic comparison demonstrated a higher tricuspid regurgitant velocity in those with a high PVR (3.4 vs 3.0 m/s; p = 0.046). FRI revealed proportionately fewer large vessels as a proportion of the vasculature in the patients without PH and elevated PVRs. Among patients without PH, PVR was associated with increased mortality. In conclusion, patients with IPF without PH but a high PVR appear to be a distinct phenotype with a prognosis between those with and without PH, likely reflecting the continuum of vascular dysfunction. The basis for this unique hemodynamic profile could not be definitively discerned although FRI suggested an aberrant anatomical vascular response.

Exercise and pulsatile pulmonary vascular loading in chronic thromboembolic pulmonary disease.

Chronic thromboembolic pulmonary disease (CTEPD) is characterized by organized nonresolving thrombi in pulmonary arteries (PA). In CTEPD with pulmonary hypertension (PH), chronic thromboembolic PH (CTEPH), early wave reflection results in abnormalities of pulsatile afterload and augmented PA pressures. We hypothesized that exercise during right heart catheterization (RHC) would elicit more frequent elevations of pulsatile vascular afterload than resistive elevations in patients with CTEPD without PH. The interdependent physiology of pulmonary venous and PA hemodynamics was also evaluated. Consecutive patients with CTEPD without PH (resting mean PA pressure ≤20 mmHg) undergoing an exercise RHC were identified. Latent resistive and pulsatile abnormalities of pulmonary vascular afterload were defined as an exercise mean PA pressure/cardiac output >3 WU, and PA pulse pressure to PA wedge pressure (PA PP/PAWP) ratio >2.5, respectively. Forty-five patients (29% female, 53 ± 14 years) with CTEPD without PH were analyzed. With exercise, 19 patients had no abnormalities (ExNOR), 26 patients had abnormalities (ExABN) of pulsatile (20), resistive (2), or both (4) elements of pulmonary vascular afterload. Exercise elicited elevations of pulsatile afterload (53%) more commonly than resistive afterload (13%) (p < 0.001). ExABN patients had lower PA compliance and higher pulmonary vascular resistance at rest and exercise and prolonged resistance-compliance time product at rest. The physiological relationship between changes in PA pressures relative to PAWP was disrupted in the ExABN group. In CTEPD without PH, exercise RHC revealed latent pulmonary vascular afterload elevations in 58% of patients with more frequent augmentation of pulsatile than resistive pulmonary vascular afterload.

Predictors Associated With Prognosis of Patients on the Waiting List for Heart Transplantation.

Aim To identify predictors associated with the prognosis of patients on the heart transplant waiting list (HTWL) corresponding to UNOS class 2.Material and methods A HTWL database for 2010-2021 was retrospectively evaluated. The study included patients (n=162) who at the time of inclusion into the HTWL met UNOS class 2 and reached the endpoint of death, heart transplantation (HT), or exclusion from the HTWL due to an improvement of their condition. Mean age was 48±13 (from 11 to 67) years, 80% (n=130) were men, and body weight index was 24.9±4.4 kg/m2. Patients were divided into two groups: group 1 (n=131), patients who left the HTWL (improvement of CHF functional class) and patients who maintained the UNOS class 2 until HT; group 2 (n=31), patients who transferred from UNOS class 2 to UNOS class 1B/1A or died while on the HTWL.Results Patients of group 2 had lower systolic BP compared to patients of group 1 (100±17, mm Hg vs. 107±17 mm Hg, respectively, p=0.03). In group 1 compared to group 2, there was a higher proportion of patients with obesity, 29 (22%) vs. 1 (3%) (p=0.02). Laboratory blood tests: absolute lymphocyte count (2.0±0.7×109/L and 1.6±0.9×109/L, p=0.03), serum albumin (42±5 g/l and 40±6 g/l, p=0.03), red cell distribution width (RDW) (16±4% and 18±4%, p=0.01); sodium concentration (139±4 and 136±4 mmol/l, p=0.009). Patients from group 2 had a higher pulmonary vascular resistance (PVR) (4.0±2.4 Wood units vs. 3.2±1.4 Wood units, p=0.01) at baseline. A predictive model (p&lt;0.001) was developed to determine a probability of prognosis in HTWL. The model sensitivity was 75% and the specificity was 67%. High PVR was a predictor that worsened the prognosis in HTWL; higher serum concentrations of sodium and albumin increased the probability of a favorable outcome in HTWL.Conclusion During the period of waiting for HT, 19% of patients that met UNOS class 2 experienced deterioration of their condition (transitioned to UNOS 1) or died. The most important predictors for a better outcome in patients on HTWL who meet UNOS class 2 were higher serum levels of sodium and albumin and low pulmonary vascular resistance.

Usefulness of PREDIC3T Case Type Risk Category in the CRISP Registry

Procedural risk in Congenital Cardiac Catheterization (PREDIC3T) was recently reported as the contemporary procedure-type risk metric by the Congenital Cardiac Catheterization Project on Outcomes (C3PO) registry. The usefulness of this metric has not been evaluated elsewhere. The CRISP registry of Congenital Cardiovascular Interventional Study Consortium (CCISC) data set was analyzed. The study period was 14years (2009 to 2022). The primary outcome was significant adverse event (SAE). Cases were assigned to the 6 PREDIC3T risk categories. Univariate and multivariable logistic regression models were used to evaluate the association between PREDIC3T and the primary outcome. The model discriminative performance was evaluated by the c-statistic. In a total of 64,419 enrolled cases, PREDIC3T case types were assigned in 59,822 cases (93%). The frequency for PREDIC3T category was 0=7,494 (12.5%), 1=16,932 (28.3%), 2=17,023 (28.5%), 3=9,885 (16.5%), 4=4,403 (7.4%), and 5=4,085 (6.8%). SAE was observed in 2,474 cases (4.1%). The SAE rates for category were 0=1.0%, 1=2.3%, 2=4.0%, 3=6.2%, 4=8.2%, and 5=9.0%. In a multivariable model, PREDIC3T case type risk category (odds ratios for category: 0=0.49, 1=1.00, 2=1.40, 3=2.06, 4=2.79, and 5=3.15; p <0.001) were significantly associated with SAE (c-statistic of 0.707) after adjusting for age, preprocedural inotropic support and systemic illness, low systemic saturation, high pulmonary vascular resistance, and the use of general anesthesia. The PREDIC3T case type risk category was associated with the risk of SAE in the CRISP registry data set and appeared to be a useful procedural risk classification tool.

Abstract 13407: Long-Term Outcomes of Chronic Thromboembolic Pulmonary Hypertension Treated With Pulmonary Endarterectomy and Balloon Pulmonary Angioplasty: Results From the Japanese Prospective CTEPH Registry (CTEPH AC Registry)

Introduction: In Japan, balloon pulmonary angioplasty (BPA) is more commonly adopted in patients with chronic thromboembolic pulmonary hypertension (CTEPH) compared to European countries or US due to their expertise. However, there are no reports from regions where BPA is extensively practiced. Aims: This study aims to explore the treatment outcomes in Japan. Methods: The study involved a nationwide, prospective, observational registry that included all patients diagnosed with CTEPH from August 2018 to February 2023 in Japan. Patients were categorized as naïve or prevalent cases based on their previous reperfusion therapy (pulmonary endarterectomy and/or BPA) at the time of enrollment. Each case was further classified as operable or BPA group. The primary outcome measure was morbidity and mortality (MM) events since enrollment, which comprised all-cause death, CTEPH-related hospitalization, rescue reperfusion therapy, initiation of parenteral pulmonary vasodilators, and worsening of CTEPH characterized by a decrease in 6-minute walk distance greater than 15% and deterioration of the WHO functional class. Results: A total of 1194 patients, including naïve cases (operable: n = 54, BPA: n = 293) and prevalent cases (operable: n=125, BPA: n = 532), were identified. Among the naïve cases, there were no significant differences in age or disease severity between the operable and BPA groups. Among the prevalent cases, the BPA group was older (63 ± 13 vs. 58 ± 14 years old, p &lt; 0.0001) and had higher pulmonary vascular resistance (364 ± 279 vs. 293 ± 206 dyne*sec/cm5, p = 0.001) compared to the operable group. The results of primary outcome measures were comparable between both the groups (Figure). Conclusions: BPA as well as pulmonary endarterectomy is considered as safety and effective therapeutic option in CTEPH patients.

Abstract 14112: Reproductive Risk Factors and Pulmonary Arterial Hypertension in Women

Introduction: Pulmonary arterial hypertension (PAH) is a devastating disease that affects women more often than men. The female predominance has increased over time; recent U.S. cohorts demonstrate a female:male ratio of nearly 4:1. The role of women-specific risk, such as reproductive exposures, is poorly understood in the pathophysiology of PAH. Hypothesis/Goals: We hypothesize that reproductive exposures influence the development and/or severity of PAH in women. We sought to investigate the role of reproductive factors and contribution of exogenous estrogen exposures to PAH onset and severity. Methods: Using reproductive history/exposure questionnaires from 390 women with PAH, enrolled in both the PAH Biobank and United States Pulmonary Hypertension Scientific Registry, we conducted linear regression analyses to assess the association between individual disease severity variables, as well as REVEAL Lite 2.0 scores, and patient reported reproductive variables as the predictors (Table 1). We adjusted for potential confounders including age, race, BMI, and PAH sub-group (idiopathic, heritable, or associated). Results: We found that younger menopause age, less than or equal to 40 years old, associates with a lower cardiac index (CI) at diagnosis [β -0.57, p 0.02]. The association persisted when controlling for use of hormone replacement therapy (HRT). There was a trend toward lower CI in those with menopause age of 41 to 50 years old, but not statistically significant [p 0.10]. Ever use of HRT was associated with a higher pulmonary vascular resistance [β 1.6, p 0.05] and lower CI [β -0.28, p 0.01] at diagnosis. Conclusion: Premature menopause (age less than 40 years) and ever use of HRT associate with worse hemodynamics at diagnosis in women with PAH, including lower cardiac index. Further investigations into reproductive exposures may offer an opportunity for more comprehensive risk factor screening and modification by physicians treating PAH patients.

Impact of Optimal Fenestration Size on Outcomes of High-Risk Fontan Patients.

We aimed to analyze mid-term outcomes of the fenestrated Fontan procedure, focusing on the fenestration size. We retrospectively reviewed the outcomes of the fenestrated Fontan procedure. Among 165 patients who underwent the Fontan procedure from 2011 to 2021, fenestration was created in 27 patients with the highest risks, including those with hypoplastic left heart syndrome, hypoplastic pulmonary arteries, heterotaxy syndrome with high pulmonary vascular resistance, and pulmonary arterial pressure >15 mm Hg. The patients underwent the procedure at a median age of three years (body weight, 11.4 kg; body surface area, 0.54 m2). Fenestration sizes were 3.5 to 5 mm. Spontaneous fenestration closure occurred within one year postoperatively in nine patients. Among them, three experienced Fontan failure, necessitating refenestration. Although fenestration size did not differ, the size corrected by body surface area at the time of surgery was smaller in patients with fenestration closure (6.4 mm/m2 vs 8.3 mm/m2, P < .05). Patients with a fenestration <7 mm/m2 were more likely to have fenestration closure within one year postoperatively, and those with a fenestration >9 mm/m2 were more likely to have severe desaturation and require home oxygen therapy following discharge. Spontaneous fenestration closure affected the frequency of Fontan complications. A very small fenestration size corrected by body surface area was a significant risk factor for spontaneous closure. Conversely, a very large fenestration size corrected by body surface area resulted in severe desaturation. The optimal fenestration size to prevent early spontaneous closure and severe desaturation is approximately 8 mm/m2.

Supracardiac Total Anomalous Pulmonary Venous Connection in Adolescence.

Background Supracardiac total anomalous pulmonary communication (TAPVC) constitutes a rare congenital cardiac anomaly. Most babies with supracardiac TAPVC are diagnosed in infancy and undergo complete surgical repair during infancy. Delayed presentation of supracardiac TAPVC is rare, and the surgical outcomes are not well known. This retrospective study was conducted to determine the presentation and surgical outcome of supracardiac TAPVC among adolescents, which constitutes an extremely rare subgroup of TAPVC. Methodology This retrospective analysis was conducted among 15 adolescent patients with supracardiac TAPVC who underwent surgical repair in the cardiothoracic surgery department of a tertiary care center in India. This study aimed to assess the intraoperative, postoperative, immediate, early, and late outcomes of adolescent patients with supracardiac TAPVC who had undergone surgical repair between 2010 and 2014 in a tertiary care center in India. Results The study included 15 patients with a diagnosis of isolated supracardiac TAPVC. A mild degree of cyanosis was present in eight patients, recurrent episodes of lower respiratory tract infections were present in five patients, and dyspnea (New York Heart Association I/II) was noted in 12 patients. Mean oxygen saturation was 92% (range = 85-93%), and mean pulmonary artery pressure was 24 mmHg (range = 15-50 mmHg). After median stenotomy, a wide anastomosis was made between the common pulmonary venous chamber and the posterior wall of the left atrium. A fenestration was made in the Dacron patch in three patients who had raised pulmonary vascular resistance (PVR) preoperatively. Twelve patients were weaned off cardiopulmonary bypass (CPB) with minimal inotropic support. Three patients who had high preoperative PVR had difficulty in weaning from CPB. The mean CPB and cross-clamp time was 75 ± 12 minutes and 58 ± 9 minutes, respectively. Atrial fibrillation was noted in five (33.3%) patients in the early postoperative period, and three (20%) patients had pulmonary artery hypertensive crises postoperatively. There was no superficial or deep sternal wound infection in the postoperative period. Mild and moderate right ventricular dysfunction was present in four (26.67%) and two (13.3%) patients, respectively, in the postoperative period. On two-dimensional echocardiography during follow-up at the end of one year, there was no gradient across the anastomosis, and pulmonary artery pressure was normal in all patients. Conclusions Surgical repair of supracardiac TAPVC in adolescence has an excellent outcome. Survival of patients with supracardiac TAPVC until adolescence depends on the presence of a dilated vertical vein and a large atrial septal defect facilitating unobstructed pulmonary venous flow. The aim of the surgical repair should be to create a wide anastomosis between the left atrium and the pulmonary venous chamber which should be bigger than the size of the mitral valve orifice indexed to the body surface area as it would amount to no or negligible anastomotic gradient postoperatively.

Factors influencing pulmonary arterial pressure in three related patients with Cantú syndrome: glyburide may provide precision care

A range of pulmonary arterial pressures was observed in three related patients with Cantú syndrome. The incident patient developed a moderately high pulmonary vascular resistance. Several factors influenced the severity of his pulmonary vascular disease and the events, which ultimately resulted in his death. However, he had an acute improvement in blood pressure and respiratory support after a single dose of glyburide when he was critically ill. The father and sister of the incident patient have evidence of mildly increased pulmonary arterial pressure with normal pulmonary vascular resistance. They are being treated with glyburide to potentially decrease the high cardiac output associated with a gain in KATP channel function. Additional experience with glyburide or other KATP channel inhibitors is needed to determine the most appropriate agent, dose, time, and duration of treatment for patients with Cantú syndrome.

Mid-Systolic Notching of the Pulmonary Valve Doppler Signal is Highly Associated with Pulmonary Hypertension.

Mid-systolic notching (MSN) of the pulmonary valve Doppler signal represents a reflected systolic pressure wave from the pulmonary vasculature and is often seen in pulmonary hypertension (PH). We hypothesize that MSN is associated with a higher pulmonary vascular resistance (PVR) and mean pulmonary artery pressure (mPAP), and a diagnosis of PH in pediatric patients. This was a retrospective study of patients ≤ 18years who had an echocardiogram obtained ≤ 30days before catheterization for suspected PH. MSN was defined as an indentation in the initial two thirds of the systolic Doppler signal. PH was defined as mPAP > 20mmHg and PVR ≥ 3.0 Wu m2. Subgroups (MSN vs. normal) were compared. Receiver operator characteristic determined a continuous variable's discriminatory ability for a diagnosis of PH. Reproducibility of MSN was assessed. In total, 90 patients (73 with congenital heart disease) were included, of which 36 had MSN and 54 were normal. MSN patients were more likely to have PH, and had significantly higher mPAP, PVR, and lower pulmonary stroke volume. The presence of MSN had good discriminatory ability for PH diagnosis. The presence of MSN had high specificity (96%) for PH, whereas sensitivity was lower (54%). Reproducibility was 100% for MSN. MSN is a simple, highly reproducible echocardiographic metric associated with higher mPAP and PVR. When present, there is a high likelihood a diagnosis of PH confirmed by catheterization. Incorporation of MSN into imaging protocols may be useful. MSN appears worthy of further investigation in pediatric patients with suspected PH.

Switching the Left and the Right Hearts: A Novel Bi-ventricle Mechanical Support Strategy with Spared Native Single-Ventricle.

End-stage Fontan patients with single-ventricle (SV) circulation are often bridged-to-heart transplantation via mechanical circulatory support (MCS). Donor shortage and complexity of the SV physiology demand innovative MCS. In this paper, an out-of-the-box circulation concept, in which the left and right ventricles are switched with each other is introduced as a novel bi-ventricle MCS configuration for the "failing" Fontan patients. In the proposed configuration, the systemic circulation is maintained through a conventional mechanical ventricle assist device (VAD) while the venous circulation is delegated to the native SV. This approach spares the SV and puts it to a new use at the right-side providing the most-needed venous flow pulsatility to the failed Fontan circulation. To analyze its feasibility and performance, eight SV failure modes have been studied via an established multi-compartmental lumped parameter cardiovascular model (LPM). Here the LPM model is experimentally validated against the corresponding pulsatile mock-up flow loop measurements of a representative 15-year-old Fontan patient employing a clinically-approved VAD (Medtronic-HeartWare). The proposed surgical configuration maintained the healthy cardiac index (3-3.5l/min/m2) and the normal mean systemic arterial pressure levels. For a failed SV with low ejection fraction (EF = 26%), representing a typical systemic Fontan failure, the proposed configuration enabled a ~ 28mmHg amplitude in the venous/pulmonary waveforms and a 2mmHg decrease in the central venous pressure (CVP) together with acceptable mean pulmonary artery pressures (17.5mmHg). The pulmonary vascular resistance (PVR)-SV failure caseprovided a ~ 5mmHg drop in the CVP, with venous/pulmonary pulsatility reaching to ~ 22mmHg. For the high PVR failure case with a healthy SV (EF = 44%) pulmonary hypertension is likely to occur as expected. While this condition is routinely encountered during the heart transplantation and managed through pulmonary vasodilators a need for precise functional assessment of the spared failed-ventricle is recommended if utilized in the PVR failure mode. Comprehensive in vitro and in silico results encourage this novel concept as a low-cost, more physiological alternative to the conventional bi-ventricle MCS pending animal experiments.

Pulmonary Hypertension in Left Ventricular Valvular Diseases: A Comprehensive Review on Pathophysiology and Prognostic Value.

Left ventricular (LV) valvular diseases, make up one of the most common etiologies for pulmonary hypertension (PH), and it is not well understood how and at which degree it affects prognosis. The aim of the present study was a comprehensive review of the pathophysiologic mechanism of PH in patients with LV valvular diseases and the prognostic value of baseline and post-intervention PH in patients undergoing interventional treatment. The pathophysiology of PH in patients with LV valvular diseases involves gradual elevation of left ventricular filling pressure and left atrial pressure, which are passively transmitted to the pulmonary circulation and raise pulmonary artery systolic pressure (PASP). A long-lasting exposure to elevated PASP progressively leads to initially functional and thereafter irreversible structural changes in the pulmonary vasculature, leading up to high pulmonary vascular resistance. Surgical treatment of severe LV valvular diseases is highly effective in patients without resting PH or those with exercise-induced PH (EIPH) before intervention. In the case of pre-operative PH, successful interventional therapy decreases PASP, but the post-operative cardiac and all-cause mortality remain higher compared to patients without pre-operative PH. Hence, it is of paramount importance to detect patients with severe LV valvulopathies before the development of PH, since they will get greater benefits from early intervention.

Pulmonary Vasodilator Therapy in Pediatric Patients on Ventricular Assist Device Support: A Single-Center Experience and Proposal for Use.

Pediatric precapillary pulmonary hypertension can develop in response to systemic atrial hypertension. Systemic atrial decompression following ventricular assist device (VAD) implantation may not sufficiently lower pulmonary vascular resistance (PVR) to consider heart transplant candidacy. Prostacyclins have been used in adult VAD patients with success, but pediatric data on safety and efficacy in this population are limited. We sought to describe our center's experience to show its safety and to present our current protocol for perioperative use. We reviewed our use of prostacyclin therapy in pediatric patients on VAD support with high PVR from 2016 to 2021. Of the 17 patients who met inclusion, 12 survived to transplant and 1 is alive with VAD in situ. All patients survived posttransplant. With continuous intravenous (IV) epoprostenol or treprostinil therapy, there were no bleeding complications or worsening of end-organ function. A significant reduction was observed in vasoactive inotropic scores by 49% in the first 24 hours post-prostacyclin initiation. The proportion of patients surviving to transplant in this high-risk cohort is favorable. In conclusion, prostacyclins may be safe to use in patients with elevated PVR as part of their VAD and transplant course and may provide a transplant option in those otherwise not candidates.