Troponin T Mutation as a Cause of Left Ventricular Systolic Dysfunction in a Young Patient with Previous Surgical Correction of Aortic Coarctation.


Journal

Biomolecules
ISSN: 2218-273X
Titre abrégé: Biomolecules
Pays: Switzerland
ID NLM: 101596414

Informations de publication

Date de publication:
06 05 2021
Historique:
received: 17 04 2021
revised: 01 05 2021
accepted: 01 05 2021
entrez: 2 6 2021
pubmed: 3 6 2021
medline: 23 9 2021
Statut: epublish

Résumé

Coarctation of the aorta is a leading cause of morbidity and mortality among adults with congenital heart disease (ACHD). Lifelong surveillance is mandatory to screen for possible long-term cardiovascular events. Left ventricular systolic dysfunction has been reported in association with recoarctation, and association with dilated cardiomyopathy (DCMP) is very rare. Herein, we report the case of a 19-year-old boy with coarctation of the aorta who complained of mild exertional dyspnea. Cardiac magnetic resonance revealed a moderately dilated, hypokinetic left ventricle (LV), with mildly reduced EF (45%), and residual isthmic coarctation was excluded. Genetic tests revealed a heterozygous missense variant in TNNT2 (NM_001001430.2): c.518G>A (p. Arg173Gln). This case highlights the role of careful history taking: a family history of cardiomyopathy should not be overlooked even when the clinical setting seems to suggest a predisposition to hemodynamic factors for LVSD.

Identifiants

pubmed: 34066613
pii: biom11050696
doi: 10.3390/biom11050696
pmc: PMC8148585
pii:
doi:

Substances chimiques

TNNT2 protein, human 0
Troponin T 0

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

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Auteurs

Martina Caiazza (M)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Michele Lioncino (M)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Emanuele Monda (E)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Francesco Di Fraia (F)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Federica Verrillo (F)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Roberta Pacileo (R)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Federica Amodio (F)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Marta Rubino (M)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Annapaola Cirillo (A)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Adelaide Fusco (A)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Emanuele Romeo (E)

Adult Congenital Heart Disease Unit, Department of Cardiology, Monaldi Hospital, 80131 Naples, Italy.

Alessandra Scatteia (A)

Division of Cardiology "Villa dei Fiori" Hospital, Acerra, 80011 Naples, Italy.

Santo Dellegrottaglie (S)

Division of Cardiology "Villa dei Fiori" Hospital, Acerra, 80011 Naples, Italy.

Paolo Calabrò (P)

Division of Clinical Cardiology, A.O.R.N. "Sant'Anna e San Sebastiano", Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", 80131 Napoli, Italy.

Berardo Sarubbi (B)

Adult Congenital Heart Disease Unit, Department of Cardiology, Monaldi Hospital, 80131 Naples, Italy.

Anwar Baban (A)

Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, 00165 Rome, Italy.

Giulia Frisso (G)

Department of Molecular Medicine and Medical Biotechnology, Federico II University of Naples, CEINGE Scarl-Advanced Biotechnologies, 80131 Naples, Italy.

Maria Giovanna Russo (MG)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.

Giuseppe Limongelli (G)

Inherited and Rare Cardiovascular Diseases, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Monaldi Hospital, 80131 Naples, Italy.
Institute of Cardiovascular Sciences, University College of London and St. Bartholomew's Hospital, London WC1E 6DD, UK.

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