Background: Left ventricular non-compaction (LVNC) cardiomyopathy is a persistence of abnormal foetal myocardium and is a rare cause of cardiomyopathy in the peripartum period. Unlike other causes of peripartum cardiomyopathy which typically improve, LVNC has significant long-term personal and family implications and needs lifelong follow-up.
Case Summary: We describe a unique case of a 30-year-old woman who developed cardiomyopathy in the peripartum period which was revealed on cardiovascular magnetic resonance imaging to be due to occult LVNC. Our patient also had Ebstein's anomaly, which is a known LVNC association.
Discussion: Cardiomyopathy in the peripartum period can be a decompensation of previously asymptomatic subclinical cardiomyopathy. It is important to assess for LVNC in patients presenting with this. Cardiovascular magnetic resonance imaging is the gold-standard imaging modality and allows accurate diagnosis of LVNC, associated structural complications and rare associations such as Ebstein's anomaly. Left ventricular non-compaction is irreversible and has implications for patients and their family members.
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http://dx.doi.org/10.1093/ehjcr/ytab088 | DOI Listing |
Int J Cardiol Congenit Heart Dis
September 2024
Adult Congenital Heart Diseases Unit, Royal Brompton Hospital, London, UK.
Peripartum cardiomyopathy (PPCM) is a rare, but serious condition, with a non-negligible risk of adverse events. Several risk factors for PPCM have been individuated over the years, including Afro-American ethnicity, preeclampsia, advanced maternal age, genetic predisposition, multiparity, twin pregnancy, obesity, smoking and diabetes. However, PPCM pathophysiology is still poorly understood, thus making it challenging to develop disease specific therapies.
View Article and Find Full Text PDFJACC Case Rep
December 2024
Division of Cardiology, Baylor Scott and White Health-Temple, Temple, Texas, USA.
Management of peripartum cardiomyopathy and cardiogenic shock often presents a significant clinical challenge. These patients are frequently best served at a specialized center with access to cardiac anesthesia, maternal-fetal medicine, and cardiac intensivists. Planning for delivery involves a plan for anesthesia and management of hemodynamic changes during the postoperative period.
View Article and Find Full Text PDFHeart Fail Rev
December 2024
Cardiovascular Services Department, Queen Elizabeth Hospital, Martindales Road, Bridgetown, Barbados.
Peripartum cardiomyopathy is an idiopathic and nonischemic systolic dysfunction with onset toward the end of pregnancy and up to 5 months postpartum. Its clinical phenotype overlaps with pregnancy-associated cardiomyopathy rendering both a continuum of the same disease. Incidence varies geographically and is highest in areas where risk factors are prevalent.
View Article and Find Full Text PDFArch Clin Cases
December 2024
Department of Obstetrics and Gynaecology, Ipswich Hospital, East Suffolk and North Essex Foundation NHS Trust, Suffolk, UK.
Peripartum cardiomyopathy (PPCM) is an uncommon life-threatening condition that is characterized by heart failure with reduced ejection fraction during late pregnancy or within 5 months of postpartum in the absence of other causes of heart failure. Despite advances in managing PPCM, the pathophysiology of it is still poorly understood. This article reviews the diagnostic challenges and management of PPCM, specifically highlighting a rare presentation of PPCM characterized by oxygen desaturation alone.
View Article and Find Full Text PDFMol Genet Metab Rep
December 2024
New Leaf Center Clinic for Special Children, 16014 East Chestnut Street Mount Eaton, OH, USA.
Background: Propionic acidemia (PA) is an inborn error of metabolism (IEM) that typically presents in the newborn. The Amish of North America have an increased prevalence of PA due to a founder variant in the gene. The Amish PA phenotype is variable, and some individuals remain asymptomatic and undiagnosed until adulthood.
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