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An unusual cause of heart failure in postoperative tetralogy of Fallot.

ABSTRACT: The long-term results of transatrial transpulmonary tetralogy of Fallot (TOF) repair have been excellent. Progressive pulmonary regurgitation and consequent right ventricular (RV) dilatation are the most common long-term sequel of definitive repair in childhood. Overt systemic venous congestion after TOF repair is limited to the rare setting where RV dysfunction sets in due to deferred surgery or progressive arrhythmia. Here, we report a unique case of right heart failure from an unexpected etiology, 28 years after TOF repair. Cardiac catheterization confirmed findings of elevated right heart pressures. Magnetic resonance imaging showed obliteration of the RV apex with late gadolinium enhancement of the right ventricular apical endomyocardium.

SUBMITTER: Singh G

PROVIDER: S-EPMC9802613 | biostudies-literature | 2022 May-Jun

REPOSITORIES: biostudies-literature

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An unusual cause of heart failure in postoperative tetralogy of Fallot.

Singh Gurbhej G Gopalakrishnan Arun A Sivasankaran Sivasubramonian S

Annals of pediatric cardiology 20220501 3

The long-term results of transatrial transpulmonary tetralogy of Fallot (TOF) repair have been excellent. Progressive pulmonary regurgitation and consequent right ventricular (RV) dilatation are the most common long-term sequel of definitive repair in childhood. Overt systemic venous congestion after TOF repair is limited to the rare setting where RV dysfunction sets in due to deferred surgery or progressive arrhythmia. Here, we report a unique case of right heart failure from an unexpected etio ...[more]

PMID: 36589660

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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.

Dataset Information

An unusual cause of heart failure in postoperative tetralogy of Fallot.

Publications

An unusual cause of heart failure in postoperative tetralogy of Fallot.

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