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Heart Failure in Tetralogy of Fallot due to Associated Hypertrophic Obstructive Cardiomyopathy: A Lesson to Learn.

ABSTRACT: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Heart failure (HF) in a case of uncomplicated TOF is uncommon but can occur under special circumstances. TOF associated with hypertrophic obstructive cardiomyopathy (HOCM) is a very rare combination of anomalies, and very few cases have been reported in the literature. Here, we report the case of a 2-month-old male infant who presented to us with central cyanosis and features of HF. He was worked up and found to have TOF with HOCM and advised surgical correction. Hence, we propose that HOCM is also one factor which can precipitate HF in a patient of TOF along with the classical causes mentioned in the literature. Furthermore, the left ventricular outflow tract obstruction of HOCM in a patient of TOF has an inverse relation with the degree of cyanosis.

SUBMITTER: Kodliwadmath A

PROVIDER: S-EPMC8230164 | biostudies-literature | 2021 Jan-Mar

REPOSITORIES: biostudies-literature

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Heart Failure in Tetralogy of Fallot due to Associated Hypertrophic Obstructive Cardiomyopathy: A Lesson to Learn.

Kodliwadmath Ashwin A Shrivastava Yash Y Duggal Bhanu B Khanra Dibbendhu D Nanda N N

Journal of cardiovascular echography 20210101 1

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Heart failure (HF) in a case of uncomplicated TOF is uncommon but can occur under special circumstances. TOF associated with hypertrophic obstructive cardiomyopathy (HOCM) is a very rare combination of anomalies, and very few cases have been reported in the literature. Here, we report the case of a 2-month-old male infant who presented to us with central cyanosis and features of HF. He was worked up and found to have ...[more]

PMID: 34221884

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Project description:BackgroundThere are limited data on the prevalence, pathophysiology, and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy and advanced heart failure.Methods and resultsTo assess the clinical significance of measured cardiopulmonary hemodynamics in hypertrophic cardiomyopathy patients with heart failure, we retrospectively assessed right heart catheterization data in 162 consecutive patients with outflow tract gradients (median [interquartile range], 90 mm Hg [70-110 mm Hg]), 59±11 years old, and 49% men, predominately New York Heart Association class III/IV status. Pulmonary hypertension (mean pulmonary artery pressure, ≥25 mm Hg) was present in 82 patients (51%), including 29 (18%) regarded as moderate-severe (mean pulmonary artery pressure, ≥35 mm Hg) and 28 (34%) also had increased pulmonary vascular resistance >3.0 WU. The pulmonary artery wedge pressure was ≤15 mm Hg in 54%, indicating that left atrial hypertension was absent in a majority of patients. Notably, 9 patients (11%) met hemodynamic criteria for precapillary pulmonary hypertension (mean pulmonary artery pressure, ≥25 mm Hg; pulmonary vascular resistance, >3.0 WU; pulmonary artery wedge pressure, ≤15 mm Hg). Over a median follow-up of 327 days (90-743 days) after surgical myectomy (or alcohol septal ablation), 92% and 95% of patients with or without preoperative pulmonary hypertension, respectively, were asymptomatic or mildly symptomatic. One postoperative death occurred in a 59-year-old woman with acute respiratory failure and mean pulmonary artery pressure of 65 mm Hg.ConclusionsPulmonary hypertension was common in obstructive hypertrophic cardiomyopathy patients with advanced heart failure. Although possibly a contributor to preoperative heart failure, pulmonary hypertension did not significantly influence clinical and surgical outcome. Notably, a novel patient subgroup was identified with resting invasive hemodynamics consistent with pulmonary vascular disease.

Project description:BackgroundMicroRNAs (miRNAs) are a class of regulatory RNAs that regulate gene expression post-transcriptionally. Little, however, is known on the expression profile of circulating miRNAs in Tetralogy of Fallot (TOF) patients late after surgical repair. In this study, we aimed to identify the specific patterns of circulating miRNAs in blood of patients with repaired, non-syndromic TOF and to assess whether these specific miRNAs may be useful to differentiate patients with and without heart failure.MethodsSurePrint™ 8 × 60 K Human v16 miRNA arrays were used to determine miRNA expression profiles in 15 healthy controls and 37 patients after TOF repair of whom 3 had symptomatic right heart failure. The expression levels of selected miRNAs have been validated by quantitative reverse transcription polymerase chain reaction (RT-qPCR). Enrichment analyses of altered miRNA expression were predicted using bioinformatic tools.ResultsCompared with healthy controls, a total of 49, 58 and 77 miRNAs were found to be significantly altered in TOF patients (TOF-all), TOF patients with (TOF-HF) and without symptomatic right heart failure (TOF-noHF) (>2.0-fold change, adjusted P < 0.05), respectively. Three miRNAs namely miR-181d-5p, miR-206 and miR-625-5p were validated by RT-qPCR in all TOF groups. The area under the receiver operating characteristic curve (AUC) for miR-181d-5p, miR-206 and miR-625-5p were 0.987, 0.993 and 0.769 in TOF-all and 0.990, 0.994 and 0.749 in TOF-noHF, respectively. Moreover, expression levels of miR-625-5p, miR-1233-3p and miR-421 were lower in TOF-HF compared to TOF-noHF (P = 0.012).ConclusionsAltered expression levels of circulating miRNAs were found in TOF patients late after surgical repair and are different to those seen in the right ventricular myocardium of infants with TOF. Expression levels of miR-421, miR-1233-3p and miR-625-5p are lower in TOF patients with symptomatic right heart failure and thus may indicate disease progression in these patients.

Project description:Background: Development of advanced heart failure (HF) symptoms is the most common adverse pathway in hypertrophic cardiomyopathy (HCM) patients. Currently, there is a limited ability to identify HCM patients at risk of HF. Objectives: In this study, we present a machine learning (ML)-based model to identify individual HCM patients who are at high risk of developing advanced HF symptoms. Methods: From a consecutive cohort of HCM patients evaluated at the Tufts HCM Institute from 2001 to 2018, we extracted a set of 64 potential risk factors measured at baseline. Only patients with New York Heart Association (NYHA) functional class I/II and LV ejection fraction (LVEF) by echocardiography >35% were included. The study cohort (n = 1,427 patients) was split into three disjoint subsets: development (50%), model selection (10%), and independent validation (40%). The least absolute shrinkage and selection operator was used to select the most influential clinical variables. An ensemble of ML classifiers, including logistic regression, was used to identify patients with high risk of developing a HF outcome. Study outcomes were defined as progression to NYHA class III/IV, drop in LVEF below 35%, septal reduction procedure, and/or heart transplantation. Results: During a mean follow-up of 4.7 ± 3.7 years, advanced HF occurred in 283 (20% out of 1,427) patients. The model features included patients' sex, NYHA class (I or II), HCM type (i.e., obstructive or not), LV wall thickness, LVEF, presence of HF symptoms (e.g., dyspnea, presyncope), comorbidities (atrial fibrillation, hypertension, mitral regurgitation, and systolic anterior motion), and type of cardiac medications. The developed risk stratification model showed strong differentiation power to identify patients at advanced HF risk in the testing dataset (c-statistics = 0.81; 95% confidence interval [CI]: 0.76, 0.86). The model allowed correct identification of high-risk patients with accuracy 74% (CI: 0.70, 0.78), sensitivity 80% (CI: 0.77, 0.83), and specificity 72% (CI: 0.68, 0.76). The model performance was comparable among different sex and age groups. Conclusions: A 5-year risk prediction of progressive HF in HCM patients can be accurately estimated using ML analysis of patients' clinical and imaging parameters. A set of 17 clinical and imaging variables were identified as the most important predictors of progressive HF in HCM.

Dataset Information

Heart Failure in Tetralogy of Fallot due to Associated Hypertrophic Obstructive Cardiomyopathy: A Lesson to Learn.

Publications

Heart Failure in Tetralogy of Fallot due to Associated Hypertrophic Obstructive Cardiomyopathy: A Lesson to Learn.

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