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Case report
Isolated left ventricular apical hypoplasia in a young child
  1. H Ravi Ramamurthy1,2,
  2. Onkar Auti3,
  3. Vimal Raj4 and
  4. Kiran Viralam5
  1. 1Department of Pediatrics, Army Hospital (Research & Referral), Delhi, India
  2. 2Department of Pediatric Cardiology & Grown Up congenital heart diseases, Narayana Institute of Cardiac Sciences, Bangalore, India
  3. 3Department of Radiology, Ruby Hall Clinic, Pune, India
  4. 4Department of Radiology, Mazumdar Shaw Medical Center, Narayana Health City, Bangalore, India
  5. 5Department of Pediatric and Grown-UpCongenital HeartDiseases, Narayana Institute of Cardiac Sciences, Bangalore, India
  1. Correspondence to Dr H Ravi Ramamurthy; drhravi{at}gmail.com

Abstract

A 16-month-old, healthy, asymptomatic male child presented with a diagnosis of dilated cardiomyopathy. Cardiovascular examination and chest radiograph were normal. ECG revealed sinus rhythm, and the augmented vector left lead showed raised ST segment, T wave inversion and q waves. Echocardiography showed a globular left ventricle with notched cardiac apex, abnormal echogenicity in the left ventricular apical myocardium, single papillary muscle and normal biventricular function. Cardiac MRI scan revealed a globular left ventricle with fibrofatty changes and retraction of the apex, the papillary muscles closely approximated, and the right ventricle wrapping around the apex of the left ventricle. This is described as isolated left ventricular apical hypoplasia. Diagnosis of this rare entity can be made by MRI, and it has been diagnosed largely in adults. The pathophysiology and long-term outcomes are unknown. We characterise the echocardiography findings of this rare anomaly in a child for the first time in the literature.

  • heart failure
  • radiology (diagnostics)

https://doi.org/10.1136/bcr-2020-239297

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    Footnotes

    • Contributors RHR managed the patient, conceptualised, designed and drafted the article, analysed the clinical and echocardiography data, performed the literature search, and followed up the patient. OA and VR managed the MRI scan, analysed the MRI data, performed the literature search, gave the radiological diagnosis of the patient, contributed to drafting the manuscript, and gave final approval of the article. KV managed the patient, designed and critically revised the article, analysed the clinical and echocardiography data, performed the literature search, and gave final approval of the article. All the authors are in agreement to be accountable for the article and ensure that all questions regarding the accuracy or integrity of the article are investigated and resolved.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Parental/guardian consent obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.