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Progression of left ventricular apical hypoplasia-like restrictive cardiomyopathy with severe pulmonary hypertension: Follow-up from fetal stage.

ABSTRACT: Left ventricular (LV) apical hypoplasia is a rare restrictive cardiomyopathy subtype with an unclear pathophysiology. LV apical hypoplasia typically presents with elongated right ventricle (RV) wrapping around a truncated and spherical LV with a deficient apex (the "banana-shape" of the RV). Here we report a case of a young boy with apparent LV apical hypoplasia that developed after birth; no "banana-shaped" RV was observed during the fetal period. Moreover, suprasystemic pulmonary hypertension (PH) developed even after a mitral valve replacement was performed for progressive mitral stenosis and regurgitation at 14 months of age. He underwent surgery for the Potts shunt, a shunt between the pulmonary artery and aorta, at 13 years to secure systemic output. His PH ameliorated and his heart failure remained stable for 3 years after the operation. This case indicates that the "banana-shaped" RV seen in this condition is not always congenital but that it can form and develop after birth. Furthermore, this case supports the usefulness of the Potts shunt as a therapeutic option in patients with severe PH due to LV apical hypoplasia. <Learning objective: Left ventricular apical hypoplasia typically presents with elongated right ventricle wrapping around a truncated and spherical left ventricle with a deficient apex. However, this characteristic may not be always congenital and can also form and develop after birth. The Potts shunt, a shunt between the pulmonary artery and aorta, may be a therapeutic option in patients with severe pulmonary hypertension due to left ventricular apical hypoplasia.>.

SUBMITTER: Sato R

PROVIDER: S-EPMC8758584 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Isolated Left Ventricular Apical Hypoplasia.

Project description: Not available

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Isolated left ventricular apical hypoplasia with myocardial non-compaction: a case report.

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| S-EPMC7047060 | biostudies-literature

Left ventricular apical aneurysm in chronic Chagas cardiomyopathy-A case report.

Project description:A 56-year-old female, who immigrated to the USA from Honduras, presented with worsening shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, and decreased exercise tolerance over the previous 2 months. She was diagnosed 1 year previously with non-ischemic dilated cardiomyopathy and non-sustained monomorphic ventricular tachycardia. An implantable cardioverter defibrillator was placed. Cause for her dilated cardiomyopathy was unknown at that time. On admission, her electrocardiogram showed low voltage complexes, with frequent premature ventricular contractions. Transthoracic two-dimensional echocardiogram (2D ECHO) showed severely reduced ejection fraction of 20%, severe mitral regurgitation with left ventricular (LV) hypokinesis, and inferolateral and inferior wall akinesis. On review of her records, a contrast 2D ECHO from the previous year revealed an aneurysm of the LV apical region. Live three-dimensional (3D) ECHO on her present admission showed persistent LV apical aneurysm. Computed tomography angiogram showed no atherosclerotic lesions. Multiple episodes of non-sustained ventricular tachycardia were recorded on telemetry. Based on these findings, the diagnosis of Chagas cardiomyopathy was entertained. Serological tests for Trypanosoma cruzi antibodies were done and returned positive. We report a case of chronic Chagas cardiomyopathy that was initially missed but ultimately diagnosed based on the finding of LV apical aneurysm. <Learning objective: Chagas cardiomyopathy is becoming increasingly prevalent in non-endemic countries. One hallmark finding associated with Chagas cardiomyopathy is left ventricle apical aneurysm. We were able to identify this finding with transthoracic contrast 2D ECHO and live 3D ECHO.>.

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Project description: Not available

| S-EPMC8343557 | biostudies-literature

Diagnosis of apical hypertrophic cardiomyopathy: T-wave inversion and relative but not absolute apical left ventricular hypertrophy.

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| S-EPMC9244999 | biostudies-literature

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| S-EPMC4152850 | biostudies-literature

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