Relation Between New York Heart Association Functional Class and Objective Measures of Cardiopulmonary Exercise in Adults With Congenital Heart Disease

2019 ◽  
Vol 123 (11) ◽  
pp. 1868-1873 ◽  
Author(s):  
Bibhuti B. Das ◽  
Ming-Lon Young ◽  
Jianli Niu ◽  
Laura E. Mendoza ◽  
Kak-Chen Chan ◽  
...  
Keyword(s):  
New York ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Association ◽  
Functional Class ◽  
Objective Measures ◽  
Cardiopulmonary Exercise ◽  
New York Heart Association

scholarly journals Long‐Term Outcome of Patients With Congenital Heart Disease Undergoing Cardiac Resynchronization Therapy

2021 ◽  
Author(s):  
Peter Kubuš ◽  
Jana Rubáčková Popelová ◽  
Jan Kovanda ◽  
Kamil Sedláček ◽  
Jan Janoušek
Keyword(s):  
New York ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Association ◽  
Cardiac Resynchronization ◽  
New York Heart Association ◽  
Crt Response

Background Cardiac resynchronization therapy (CRT) is rarely used in patients with congenital heart disease, and reported follow‐up is short. We sought to evaluate long‐term impact of CRT in a single‐center cohort of patients with congenital heart disease. Methods and Results Thirty‐two consecutive patients with structural congenital heart disease (N=30) or congenital atrioventricular block (N=2), aged median of 12.9 years at CRT with pacing capability device implantation, were followed up for a median of 8.7 years. CRT response was defined as an increase in systemic ventricular ejection fraction or fractional area of change by >10 units and improved or unchanged New York Heart Association class. Freedom from cardiovascular death, heart failure hospitalization, or new transplant listing was 92.6% and 83.2% at 5 and 10 years, respectively. Freedom from CRT complications, leading to surgical system revision (elective generator replacement excluded) or therapy termination, was 82.7% and 72.2% at 5 and 10 years, respectively. The overall probability of an uneventful therapy continuation was 76.3% and 58.8% at 5 and 10 years, respectively. There was a significant increase in ejection fraction/fractional area of change ( P <0.001) mainly attributable to patients with systemic left ventricle ( P =0.002) and decrease in systemic ventricular end‐diastolic dimensions ( P <0.05) after CRT. New York Heart Association functional class improved from a median 2.0 to 1.25 ( P <0.001). Long‐term CRT response was present in 54.8% of patients at last follow‐up and was more frequent in systemic left ventricle ( P <0.001). Conclusions CRT in patients with congenital heart disease was associated with acceptable survival and long‐term response in ≈50% of patients. Probability of an uneventful CRT continuation was modest.

scholarly journals Peripartum cardiomyopathy in uncorrected tetralogy of fallot: a case report

2018 ◽  
Vol 7 (2) ◽  
pp. 748
Author(s):  
Mukund Vasantrao Phutane ◽  
Varsha Ansaram Kal ◽  
Vishal Vijayrao Patil ◽  
N. O. Bansal
Keyword(s):  
New York ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Heart Association ◽  
Fetal Outcome ◽  
Pregnancy And Delivery ◽  
New York Heart Association ◽  
Postpartum Cardiomyopathy

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart with incidence about 10% of all congenital heart disease. Natural survival to the fourth decade is extremely rare (only about 3%), but there is a tendency of increasing number of women with cyanotic congenital heart disease living 3 to 4 decades and are becoming pregnant. Because of significant physiology adaptation and changes, pregnancy and delivery process are troublesome for most unhealthy women, including those with uncorrected ToF. For ToF patients, it remains an important cause of maternal morbidity (62.5%), and even mortality (10%) and has significant effects on fetal outcome. Discussed below a case of pregnancy in a 30 year old woman with uncorrected ToF, dyspnoea class II of New York Heart Association, on 36th week pregnancy with complication of postpartum cardiomyopathy. Through dedicated medical care, patient's condition improvement can be seen. Prognosis of pregnancy in patient with uncorrected ToF is poor without optimal obstetrical and medical management.

Early Experience With Sacubitril/Valsartan in Adult Patients With Congenital Heart Disease

2019 ◽  
Vol 10 (3) ◽  
pp. 292-295 ◽  
Author(s):  
Gentian Lluri ◽  
Jeannette Lin ◽  
Leigh Reardon ◽  
Pamela Miner ◽  
Katrina Whalen ◽  
...  
Keyword(s):  
New York ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Nyha Class ◽  
Heart Association ◽  
Class Ii ◽  
Class Iii

Background: Heart failure (HF) is the leading cause of hospitalizations and death in patients with adult congenital heart disease (ACHD). Sacubitril/valsartan is a new agent in the treatment of HF, but its effects have not been assessed in ACHD. Methods: We retrospectively studied all 15 patients with ACHD at our center who were prescribed sacubitril/valsartan between June 2017 and June 2018. We assessed baseline characteristics and clinical and laboratory changes after initiation of sacubitril/valsartan. Adverse events, including renal function, medication intolerance, and worsening HF were documented. Results: The median age was 53.2 (27.6-83.6) years, with a median follow-up duration of 69 (8-419) days. At baseline, all patients had refractory HF despite guideline-directed medical therapy, with ten (67%) patients as New York Heart Association (NYHA) class II, and five (33%) patients NYHA class III. The medication was discontinued in one (7%) patient secondary to worsening kidney function. No patients reported clinical deterioration; four NYHA class III patients with complex CHD, pulmonary hypertension, and cyanosis reported significant improvement to NYHA class II. Baseline creatinine was 1.1 (0.9-1.7) and two weeks after starting sacubitril/valsartan it was 1.3 (0.8-2.5, P = .22). Conclusions: Sacubitril/valsartan seems to be well tolerated in patients with ACHD who present with refractory HF symptoms. Patients with complex CHD associated with cyanosis and pulmonary hypertension could benefit the most, but larger studies are needed to assess the safety as well as the effectiveness of sacubitril/valsartan in this patient population.

scholarly journals Cardiopulmonary Exercise Testing—A Valuable Tool, Not Gatekeeper When Referring Patients With Adult Congenital Heart Disease for Transplant Evaluation

2019 ◽  
Vol 10 (3) ◽  
pp. 286-291 ◽  
Author(s):  
Jonathan N. Menachem ◽  
Nosheen Reza ◽  
Jeremy A. Mazurek ◽  
Danielle Burstein ◽  
Edo Y. Birati ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Exercise Testing ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Cardiopulmonary Exercise Testing ◽  
Peak Oxygen Consumption ◽  
Cardiopulmonary Exercise ◽  
Advanced Therapies ◽  
Adult Congenital

Introduction: Treatment of patients with adult congenital heart disease (ACHD) with advanced therapies including heart transplant (HT) is often delayed due to paucity of objective prognostic markers for the severity of heart failure (HF). While the utility of Cardiopulmonary Exercise Testing (CPET) in non-ACHD patients has been well-defined as it relates to prognosis, CPET for this purpose in ACHD is still under investigation. Methods: We performed a retrospective cohort study of 20 consecutive patients with ACHD who underwent HT between March 2010 and February 2016. Only 12 of 20 patients underwent CPET prior to transplantation. Demographics, standard measures of CPET interpretation, and 30-day and 1-year post transplantation outcomes were collected. Results: Patient Characteristics. Twenty patients with ACHD were transplanted at a median of 40 years of age (range: 23-57 years). Of the 12 patients who underwent CPET, 4 had undergone Fontan procedures, 4 had tetralogy of Fallot, 3 had d-transposition of the great arteries, and 1 had Ebstein anomaly. Thirty-day and one-year survival was 100%. All tests included in the analysis had a peak respiratory quotient _1.0. The median peak oxygen consumption per unit time (_VO2) for all diagnoses was 18.2 mL/kg/min (46% predicted), ranging from 12.2 to 22.6. Conclusion: There is a paucity of data to support best practices for patients with ACHD requiring transplantation. While it cannot be proven based on available data, it could be inferred that outcomes would have been worse or perhaps life sustaining options unavailable if providers delayed referral because of the lack of attainment of CPET-specific thresholds.

An Early Canadian Experience with the Correx Automated Coring and Apical Connector Device for Aortic Valve Bypass

2016 ◽  
Vol 11 (6) ◽  
pp. 434-438
Author(s):  
Hussein A. Al-Amodi ◽  
Christopher L. Tarola ◽  
Hamad F. Alhabib ◽  
Corey Adams ◽  
Linrui Ray Guo ◽  
...  
Keyword(s):  
New York ◽  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Heart Association ◽  
Left Ventricular ◽  
Functional Class ◽  
New York Heart Association ◽  
Apical Connector ◽  
Aortic Valve Bypass

Objective Aortic valve replacement is the standard of care for severe, symptomatic aortic valve stenosis (AS); however, anatomy or preexisting comorbidities may preclude conventional or alternative transcatheter approaches. Aortic valve bypass (AVB) may be performed as a salvage procedure for the relief of symptomatic aortic stenosis in patients who are not suitable candidates for aortic valve replacement. Methods At our institution, seven patients underwent AVB using the Correx automated coring and apical connector system. All patients had severe AS with New York Heart Association functional class 3 symptoms and were not candidates for conventional or transcatheter approaches. Via a left anterolateral thoracotomy to access the descending aorta and left ventricular apex, we used the Correx system (Correx, Waltham, MA USA) to anastomose a valve conduit to the left ventricular apex proximally and the descending aorta distally. Three patients required cardiopulmonary bypass. Results In all seven patients, the automated coring and apical connector was successfully deployed. There were two in-hospital deaths in this series. Immediately postoperatively and at 3 months, there was a significant reduction in mean and peak valve gradients, and all surviving patients performed at New York Heart Association functional class 1. Conclusions Aortic valve bypass seems to be an acceptable alternative for the treatment of severe AS in high-risk patients who are not candidates for aortic valve replacement. The Correx automated system may improve the clinical applicability and surgical repro-ducibility of AVB in appropriately selected patients in which conventional or transcatheter aortic valve replacement is not a feasible options.

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