Left ventricular noncompaction presenting as peripartum cardiomyopathy

Lea, Brian; Bailey, Alison L.; Wiisanen, Matthew; Attili, Anil; Rajagopalan, Navin

doi:10.1016/j.ijcard.2011.06.073

Cited by 14 publications

(6 citation statements)

References 7 publications

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“…This genetic heterogeneity explains both the genotypic overlap of different cardiomyopathies, as well as the phenotypic overlap that might occur. There are very few case reports regarding pregnant women, known or subsequently diagnosed with ILVNC, who gave birth to children with ILVNC, but no genetic analyses were available (35,47,48). As genetic testing was not available in our case report either, we conclude that despite the existence of some clinical manifestation patterns according to the genetic mutation, given the broad genetic variability, in the absence of molecular genetic analysis, only clinical assumptions can be made regarding the causative mutation.…”

Section: Genetic Aspectsmentioning

confidence: 70%

“…The main differential diagnosis of women with ILVNC in the setting of pregnancy/postpartum period should be performed with PPC. A few cases have been reported regarding pregnant women diagnosed with PPC who simultaneously presented characteristic features of LVNC (34)(35)(36)(37). Out of a total of eight patients, none presented complete normalization of LV function at 6 months postpartum.…”

Section: Definitive and Differential Diagnosismentioning

confidence: 99%

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

et al. 2020

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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 4years-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.

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Section: Genetic Aspectsmentioning

confidence: 70%

Section: Definitive and Differential Diagnosismentioning

confidence: 99%

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

et al. 2020

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“…However, similar histological studies have not been performed in healthy women pre-or postpartum as these are limited by the requirement of cardiac biopsies and the associated risks. PPCM can have various cardiac phenotypes including ventricular dilation 1 , borderline non-compaction cardiomyopathy 38,39 , and peripartum takotsubo cardiomyopathy 40,41 , whereas normal pregnancy is associated with reversible eccentric cardiac hypertrophy 42 . This disparity may indicate that regulatory mechanisms involved in physiological cardiac remodelling during and after pregnancy could be impaired, leading to a 6 decompensated phenotype.…”

Section: Structural Changesmentioning

confidence: 99%

Pathophysiology and risk factors of peripartum cardiomyopathy

et al. 2022

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Peripartum cardiomyopathy (PPCM) is a potentially fatal form of idiopathic heart failure with varying incidences among countries and races. The cause of PPCM is uncertain but it may result from a combination of environmental and genetic factors, as well as pregnancy associated conditions such as pre-eclampsia. Animal studies suggested that impaired vascular and metabolic function may be central to the development of PPCM. Clarifying the pathogenic mechanisms is necessary to establish new therapies to improve the outcomes of patients with PPCM. Pregnancy hormones tightly coordinate a plethora of maternal adaptive responses, including haemodynamic, vascular, structural, and metabolic changes of the cardiovascular system. While pregnancy is considered to be a cardiovascular challenge, hormonal effects uniquely drive systemic insulin resistance and mostly fatty acid-dependent cardiac metabolism. In PPCM, the peripartum period is associated with profound and rapid hormonal changes that result in a brief period of disrupted cardiovascular (metabolic) homeostasis prone to secondary perturbations. This review summarizes and reflects on recent literature on the potential pathophysiological mechanisms and risk factors for PPCM with a focus on the maternal cardiovascular changes associated with pregnancy. We provide an updated framework to improve understanding of PPCM pathogenesis, which may lead to a better disease definition.

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“…Thus, it is not surprising that a subset of patients with peripartum heart failure turned out to be carriers of mutations associated with familial forms of dilated cardiomyopathies (DCM), including mutations MYH7, SCN5A, PSEN2, MYH6, TNNT2, cardiac troponin C (TNNC1), and MYBPC3 [15,16]. Previously not detected non-compaction cardiomyopathy is also present in some of these patients [17,18]. A transient condition of borderline non-compaction c ardiomyopathy resolving after functional recovery suggests that it may not be so easy to distinguish non-genetic forms like PPCM from genetically caused or at least fostered forms of peripartum heart failure [19].…”

Section: Familial Predisposition and Genetic Forms Of Pregnancy-assocmentioning

confidence: 99%

Pregnancy-associated cardiomyopathies

2014

Cardiovasc Med

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SummaryCardiovascular disease is a major cause of complications in pregnancy worldwide and the number of patients who develop cardiac problems during pregnancy is increasing.The physiological changes occurring during pregnancy and in the peripartum period provide a challenge to the cardiovascular system of all women. Understanding the morphological and functional changes in normal pregnancy is therefore important for the timely recognition of cardiovascular pathology during this vulnerable period. However, evidence-based clinical data in this field are scarce and there is a deficit in understanding general physiological and pathophysiological processes operating in the maternal heart around pregnancy.This review focuses on novel aspects of physiological and pathophysiological changes of the maternal cardiovascular system with a special focus laid on hypertensive complications in pregnancy, i.e., pre-eclampsia. A second focus is set on peripartum heart failure, especially peripartum cardiomyopathy (PPCM) which is a potentially life-threatening heart disease emerging towards the end of pregnancy or in the first postpartal months i n previously healthy women. W e present clinical and basic science data on the current state of knowledge of normal pregnancy, pre-eclampsia and PPCM and bring them into context thereby highlighting promising novel diagnostic tools and therapeutic approaches.

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Left ventricular noncompaction presenting as peripartum cardiomyopathy

Cited by 14 publications

References 7 publications

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

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

Pathophysiology and risk factors of peripartum cardiomyopathy

Pregnancy-associated cardiomyopathies

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