Isolated Left Ventricular Apical Hypoplasia: Reporting a Case With Mild Manifestations and Different Echocardiography Features

Abstract

IntroductionIsolated left ventricular apical hypoplasia is an unusual type of cardiomyopathy that presents with different clinical manifestations according to the age of the disease, ranging from no symptoms in children to congestive heart failure, pulmonary edema, or even malignant tachycardia in adults. To our knowledge, only a few cases of isolated left ventricular apical hypoplasia have been reported in Asian adults.Case PresentationHerein, we described an adult case of isolated left ventricular apical hypoplasia referred to our heart center in Isfahan, Iran in 2015 with a complaint of mild dyspnea with the absence of obvious fatty tissue in the heart’s apex and an absence of any shunt, which are common findings in patients with this phenomenon.ConclusionsPatients faced with isolated left ventricular apical hypoplasia should be monitored by echocardiography because of this disease’s possible progressive trend to life-threatening consequences.

Isolated left ventricular apical hypoplasia: Systematic review and analysis of the 37 cases reported so far

“…Other 4378 papers were excluded after checking the abstract. The remaining 29 manuscripts were used for analysis of the patients’ features and disease outcome (Table 1 )[ 8 - 29 ].…”

Apical Hypoplasia of the Left Ventricle A Very Rare Congenital Heart Disease

“…There is currently very little knowledge about what appears to be a rare congenital disease. The cause is unknown, but it may be related to inadequate LV-RV dilation, and identified a mutation of the lamin A/C gene, a gene known to be associated with other forms of cardiomyopathy, there is a clinical trial in progress to identify the genetic basis of this alteration since until now it is not associated with any race specifically [3].…”

Isolated ventricular apical hypoplasia: A report of four cases and literature review

“…Different degrees of atrioventricular regurgitation, due to the papillary muscles abnormally originating from the truncated apex, can be corrected by surgery 11 . The severely affected patients may need invasive procedures and even cardiac transplantation, particularly before the development of irreversible pulmonary hypertension 24 . However, no case of cardiac transplantation has been described, although heart or heart and lung transplantation have been suggested as potentially necessary in severe cases complicated by pulmonary hypertension 22 …”