Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review

Left ventricular non-compaction (LVNC) is a form of cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses which form a distinct “non-compacted” layer in the myocardium. It results from intrauterine arrest of the compaction process of the left ventricular myocardium....

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Main Authors: Hamida Al Hussein, Hussam Al Hussein, Valentin Stroe, Marius Harpa, Claudiu Ghiragosian, Cristina Maria Goia, Carmen Elena Opris, Horatiu Suciu
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-07-01
Series:Frontiers in Pediatrics
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fped.2020.00370/full
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spelling doaj-26bc5d55d73a4ecbbcae3ae13e69f6a42020-11-25T03:04:15ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602020-07-01810.3389/fped.2020.00370533704Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature ReviewHamida Al Hussein0Hussam Al Hussein1Valentin Stroe2Marius Harpa3Claudiu Ghiragosian4Cristina Maria Goia5Carmen Elena Opris6Horatiu Suciu7Department of Morphological Sciences, University of Medicine and Pharmacy of Târgu Mureş, Târgu Mureş, RomaniaDepartment of Cardiovascular Surgery, University of Medicine and Pharmacy of Târgu Mureş, Târgu Mureş, RomaniaDepartment of Cardiovascular Surgery, University of Medicine and Pharmacy of Târgu Mureş, Târgu Mureş, RomaniaDepartment of Cardiovascular Surgery, University of Medicine and Pharmacy of Târgu Mureş, Târgu Mureş, RomaniaDepartment of Cardiovascular Surgery, University of Medicine and Pharmacy of Târgu Mureş, Târgu Mureş, RomaniaDepartment of Cardiovascular Surgery, University of Medicine and Pharmacy of Târgu Mureş, Târgu Mureş, RomaniaDepartment of Cardiology, University of Medicine and Pharmacy of Târgu Mureş, Târgu Mureş, RomaniaDepartment of Cardiovascular Surgery, University of Medicine and Pharmacy of Târgu Mureş, Târgu Mureş, RomaniaLeft ventricular non-compaction (LVNC) is a form of cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses which form a distinct “non-compacted” layer in the myocardium. It results from intrauterine arrest of the compaction process of the left ventricular myocardium. Clinical manifestations vary from asymptomatic to heart failure (HF), arrhythmias, or thromboembolic events. We present a case of mother and son diagnosed with isolated LVNC (ILVNC). A 4-years-old male patient, diagnosed at 3 months with ILVNC, and NYHA functional class IV HF, was admitted to the Emergency Institute for Cardiovascular Diseases and Transplantation of Targu Mures, Romania, for cardiologic reevaluation, and diagnosis confirmation. ILVNC was confirmed using echocardiography, revealing a non-compaction to compaction (NC/C) ratio of > 2.7. His evolution was stationary until the age of 8 years, when severe pneumonia caused hemodynamic decompensation, and he was listed for heart transplantation (HT). The patient underwent HT at the age of 11 years with favorable postoperative outcome. Meanwhile, a 22-years-old female patient, mother of the aforementioned patient, was also admitted to our institute due to severe fatigue, dyspnea, and recurrent palpitations with multiple implantable cardioverter defibrillator (ICD) shock delivery. Extensive medical history revealed that a presumptive ILVNC diagnosis was established when she was 11 years old. She was asymptomatic until 18 years old, when 3 months post-partum, she developed NYHA functional class III HF, and subsequently underwent ICD implantation. Her diagnosis was confirmed using multi-detector computed tomography angiography, which revealed a NC/C ratio of > 3.3. ICD adjustments were carried out with a favorable evolution under chronic drug therapy. The last evaluation, at 27 years old, revealed that she was in NYHA functional class II HF. In conclusion, ILVNC, even when familial, can present different clinical pictures and therefore requires different medical approaches.https://www.frontiersin.org/article/10.3389/fped.2020.00370/fullleft ventricular non-compactionfamilialdilated cardiomyopathyheart transplantationperipartum cardiomyopathyventricular tachycardia
collection DOAJ
language English
format Article
sources DOAJ
author Hamida Al Hussein
Hussam Al Hussein
Valentin Stroe
Marius Harpa
Claudiu Ghiragosian
Cristina Maria Goia
Carmen Elena Opris
Horatiu Suciu
spellingShingle Hamida Al Hussein
Hussam Al Hussein
Valentin Stroe
Marius Harpa
Claudiu Ghiragosian
Cristina Maria Goia
Carmen Elena Opris
Horatiu Suciu
Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review
Frontiers in Pediatrics
left ventricular non-compaction
familial
dilated cardiomyopathy
heart transplantation
peripartum cardiomyopathy
ventricular tachycardia
author_facet Hamida Al Hussein
Hussam Al Hussein
Valentin Stroe
Marius Harpa
Claudiu Ghiragosian
Cristina Maria Goia
Carmen Elena Opris
Horatiu Suciu
author_sort Hamida Al Hussein
title Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review
title_short Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review
title_full Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review
title_fullStr Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review
title_full_unstemmed Different Manifestations in Familial Isolated Left Ventricular Non-compaction: Two Case Reports and Literature Review
title_sort different manifestations in familial isolated left ventricular non-compaction: two case reports and literature review
publisher Frontiers Media S.A.
series Frontiers in Pediatrics
issn 2296-2360
publishDate 2020-07-01
description Left ventricular non-compaction (LVNC) is a form of cardiomyopathy characterized by prominent trabeculae and deep intertrabecular recesses which form a distinct “non-compacted” layer in the myocardium. It results from intrauterine arrest of the compaction process of the left ventricular myocardium. Clinical manifestations vary from asymptomatic to heart failure (HF), arrhythmias, or thromboembolic events. We present a case of mother and son diagnosed with isolated LVNC (ILVNC). A 4-years-old male patient, diagnosed at 3 months with ILVNC, and NYHA functional class IV HF, was admitted to the Emergency Institute for Cardiovascular Diseases and Transplantation of Targu Mures, Romania, for cardiologic reevaluation, and diagnosis confirmation. ILVNC was confirmed using echocardiography, revealing a non-compaction to compaction (NC/C) ratio of > 2.7. His evolution was stationary until the age of 8 years, when severe pneumonia caused hemodynamic decompensation, and he was listed for heart transplantation (HT). The patient underwent HT at the age of 11 years with favorable postoperative outcome. Meanwhile, a 22-years-old female patient, mother of the aforementioned patient, was also admitted to our institute due to severe fatigue, dyspnea, and recurrent palpitations with multiple implantable cardioverter defibrillator (ICD) shock delivery. Extensive medical history revealed that a presumptive ILVNC diagnosis was established when she was 11 years old. She was asymptomatic until 18 years old, when 3 months post-partum, she developed NYHA functional class III HF, and subsequently underwent ICD implantation. Her diagnosis was confirmed using multi-detector computed tomography angiography, which revealed a NC/C ratio of > 3.3. ICD adjustments were carried out with a favorable evolution under chronic drug therapy. The last evaluation, at 27 years old, revealed that she was in NYHA functional class II HF. In conclusion, ILVNC, even when familial, can present different clinical pictures and therefore requires different medical approaches.
topic left ventricular non-compaction
familial
dilated cardiomyopathy
heart transplantation
peripartum cardiomyopathy
ventricular tachycardia
url https://www.frontiersin.org/article/10.3389/fped.2020.00370/full
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