Remarkably Still Repairable Large Aortopulmonary Window in an Adult Patient

2019 ◽  
Vol 11 (1) ◽  
pp. 117-119
Author(s):  
Panagiotis M. Zografos ◽  
Eleftherios M. Protopapas ◽  
Nikolaos I. Hakim ◽  
Chrysanthos Alexopoulos ◽  
George E. Sarris
Keyword(s):  
Heart Failure ◽  
Pulmonary Arterial Hypertension ◽  
Rare Case ◽  
Main Pulmonary Artery ◽  
Ascending Aorta ◽  
Aortopulmonary Window ◽  
Asymptomatic Adult ◽  
Pulmonary Arterial ◽  
Pulmonary Vascular Obstructive Disease ◽  
Successful Repair

Aortopulmonary window (APW) is an abnormal congenital connection between the main pulmonary artery (MPA) and the ascending aorta, with intact aortic and pulmonary valves, leading to heart failure or, if not repaired early, to pulmonary vascular obstructive disease. We report the rare case of an asymptomatic adult with an unrestrictive APW, whose pulmonary arterial hypertension was remarkably still reversible, permitting successful repair.

A prenatal diagnosis of unusual aortopulmonary communication: tubular aortopulmonary window

2021 ◽  
pp. 1-3
Author(s):  
Kadir Babaoğlu ◽  
Eviç Zeynep Başar ◽  
Rıza Türköz
Keyword(s):  
Pulmonary Artery ◽  
Rare Case ◽  
Fetal Echocardiography ◽  
Main Pulmonary Artery ◽  
Ascending Aorta ◽  
Right Aortic Arch ◽  
Aortopulmonary Window ◽  
Great Vessels ◽  
Large Tunnel

Abstract We described a very rare case of aorto-pulmonary communication with right aortic arch and crossed pulmonary artery that cannot be placed in the typical anatomic classification of aortopulmonary window. At 23 weeks gestation, fetal echocardiography revealed a large tunnel-like communication connecting the great vessels proximal to the main pulmonary artery bifurcation, rather than a classic aortopulmonary window between the ascending aorta and the main pulmonary artery.

scholarly journals A Case of Aortopulmonary Window: Asymptomatic until the First Pregnancy

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Murat Kose ◽  
Serra Ucar ◽  
Samim Emet ◽  
Timur Selcuk Akpinar ◽  
Kıvanc Yalin
Keyword(s):  
Pulmonary Hypertension ◽  
Rare Case ◽  
Ascending Aorta ◽  
Pulmonary Trunk ◽  
Cardiac Diseases ◽  
Aortopulmonary Window ◽  
Severe Pulmonary Hypertension ◽  
Adult Case ◽  
Rare Congenital Anomaly ◽  
Asymptomatic Adult

The aortopulmonary window (APW) is an abnormal communication between the ascending aorta and the pulmonary trunk in the presence of two separate semilunar valves. It is a rare congenital malformation which represents 0.1% of all congenital cardiac diseases. Herein, we report a very rare case of 27-year-old patient with unrepaired APW causing Eisenmenger syndrome and pulmonary hypertension who was asymptomatic until her first pregnancy. The median survival of uncorrected APW is 33 years. Aortopulmonary window is a very rare congenital anomaly. To our knowledge, asymptomatic adult case has not been reported until now. APW should be considered in the differential diagnosis of the severe pulmonary hypertension also in adult patients.

scholarly journals Critical Care Management of Decompensated Right Heart Failure in Pulmonary Arterial Hypertension Patients – An Ongoing Approach

2021 ◽  
Vol 7 (3) ◽  
pp. 170-183
Author(s):  
Ioan Tilea ◽  
Andreea Varga ◽  
Anca-Meda Georgescu ◽  
Bianca-Liana Grigorescu
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Intensive Care ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Care Management ◽  
Right Heart Failure ◽  
Right Heart ◽  
Continuous Increase ◽  
Pulmonary Arterial

Abstract Despite substantial advancements in diagnosis and specific medical therapy in pulmonary arterial hypertension patients’ management, this condition continues to represent a major cause of mortality worldwide. In pulmonary arterial hypertension, the continuous increase of pulmonary vascular resistance and rapid development of right heart failure determine a poor prognosis. Against targeted therapy, patients inexorable deteriorate over time. Pulmonary arterial hypertension patients with acute right heart failure who need intensive care unit admission present a complexity of the disease pathophysiology. Intensive care management challenges are multifaceted. Awareness of algorithms of right-sided heart failure monitoring in intensive care units, targeted pulmonary hypertension therapies, and recognition of precipitating factors, hemodynamic instability and progressive multisystem organ failure requires a multidisciplinary pulmonary hypertension team. This paper summarizes the management strategies of acute right-sided heart failure in pulmonary arterial hypertension adult cases based on recently available data.

Abstract 12455: Evaluation of N-Terminal Pro-B-type Natriuretic Peptide as a Therapeutic Response Biomarker in Group I Pulmonary Arterial Hypertension

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Aaron M Wolfson ◽  
Micheal L Maitland ◽  
Vasiliki Thomeas ◽  
Cherylanne Glassner ◽  
Mardi Gomberg-Maitland
Keyword(s):  
Heart Failure ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Roc Curve ◽  
Curve Analysis ◽  
Roc Curve Analysis ◽  
Goal Directed Therapy ◽  
Group I ◽  
Target Values ◽  
Pulmonary Arterial

Purpose: Goal directed management of left heart failure with an NT-proBNP target-based approach has some evidence of providing a survival benefit. To evaluate the potential utility of serial NT-proBNP measurements for goal-directed therapy in right heart failure we retrospectively assessed NT-proBNP as a predictor for survival in Group I pulmonary arterial hypertension (PAH) patients. Methods: We identified 103 Group I PAH patients from a pulmonary hypertension registry who had baseline elevated NT-proBNP prior to either the initiation or escalation of therapy and at least two serial NT-proBNP measurements. In a two-step process, we (1) estimated baseline NT-proBNP and slope (rate of change of NT-proBNP) with a linear mixed-effects model using all patient data and then (2) compared the power of serial versus single measurements in predicting survival with measured and model-derived values of baseline NT-proBNP with a Receiver Operative Characteristic (ROC) curve analysis . Survival was determined using the Kaplan-Meier methodology. Results: ROC curve analysis revealed significantly higher AUC for model-derived NT-proBNP values compared to the measured values (AUC: for baseline 0.74 vs 0.66, p= 0.009; for slope 0.78 vs 0.66, p= 0.02). Optimal cutpoints for prediction of survival on baseline NT-proBNP were 2012 (measured) vs. 1810 (model-derived) pg/mL. The optimal cutpoint for model-derived change in NT-proBNP was -0.004 log10pg/mL/month. Sensitivity, specificity, and negative predictive values for the three predictor variables were: 64%, 67%, 80% (measured baseline NT-proBNP), 61%, 80%, 81% (model-derived baseline NT-proBNP) and 73%, 57%, 85% (model-derived slope). Conclusions: In PAH patients, serial NT-proBNP measurements better predict survival than single measurements. This retrospective finding reveals that changes in NT-proBNP are associated with overall survival in PAH patients, and set initial target values for a pilot prospective study of NT-proBNP goal-directed therapy.

Abstract 14610: Renal Dysfunction in Adults With Congenital Heart Disease in Vietnam

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Ngoc Thanh Kim ◽  
Thanh Tung Le ◽  
Doan Loi Do ◽  
Thanh Huong Truong
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Atrial Fibrillation ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Renal Dysfunction ◽  
Congenital Heart ◽  
Pulmonary Arterial

Introduction: In Vietnam, knowledge about renal function in adults with congenital heart disease (CHD) is limited. Hypothesis: This study aims to estimate incidence of renal dysfunction in adults with congenital heart disease and risk factors. Methods: This is a cross-sectional study, including 365 CHD patients more than 16 years old. We collected clinical and para-clinical information, estimated glomerular filtration rate (GFR) and calculated the odds ratio (OR) for reduced GFR. Results: Totally, 52.8% patients had GFR < 90 ml/phút/1.73 m 2 . Logistic regression had confirmed the OR for GFR < 90 ml/phút/1.73 m 2 in the group > 60-years-old, the group with atrial fibrillation, the group with heart failure (based on NT-proBNP > 125 pmol/L), and the group with pulmonary arterial hypertension (based on pulmonary artery systolic pressure > 50 mmHg by echocardiography) were 6.46 (95% CI: 1.37 - 30.41), 7.58 (95% CI: 1.66 - 34.56), 2.98 (95% CI: 1.49 - 5.98) and 1.84 (95% CI: 1.02 - 3.33), respectively. Conclusions: Renal dysfunction is common in adults with CHD. Age > 60 years-old, atrial fibrillation, heart failure, and pulmonary arterial hypertension were risk factors for renal dysfunction in adults with CHD.

Sign in / Sign up

Export Citation Format

Share Document