title: Biventricular non-compaction with predominant right ventricular involvement, reduced left ventricular systolic and diastolic function, and pulmonary hypertension in a Hispanic male.
abstract: METHODS
  Male, 22 FINAL DIAGNOSIS: Cardiomyopathy Symptoms: Shortness of breath • dispnoea • chest discomfort
  
  
  METHODS
  - Clinical Procedure: Echocardiogram • cardiac MRI Specialty: Cardiology.
  
  
  OBJECTIVE
  Challenging differential diagnosis.
  
  
  BACKGROUND
  Non-compaction cardiomyopathy (NCM) is a rare congenital cardiomyopathy characterized by increased trabeculation in one or more segments of the ventricle. The left ventricle is most commonly affected. However, biventricular involvement or right ventricle predominance has also been described. Clinical features of NCM are non-specific and can range from being asymptomatic to symptoms of congestive heart failure, arrhythmia, and systemic thromboembolism.
  
  
  METHODS
  22-year-old Hispanic male presented with two month history of chest discomfort. Laboratory workup revealed an elevated brain-natriuretic-peptide of 1768 pg/ml. ECG and chest x-ray was nonspecific. Transthoracic echocardiogram revealed prominent trabeculae and spongiform appearance of the left ventricle (LV) with an ejection-fraction of 15-20%; 5 of 9 segments of the LV were trabeculated with deep intertrabecular recesses also involving the right ventricle (RV) with demonstrated blood flow in these recesses on color-doppler. The biventricular spongiform appearance was morphologically suggestive for NCM with involvement of the RV. Confirmatory cardiac MRI was performed, demonstrating excessive trabeculation of the left-ventricular apex and mid-ventricular segments. Hypertrabecularion was exhibited at the apical and lateral wall of the RV. Cardiac catheterization showed an intact cardiac vessel system. The patient was discharged on heart failure treatment and was placed on the heart transplantation list.
  
  
  CONCLUSIONS
  NCM is a unique disorder resulting in serious and severe complications. The majority of the reported cases describe the involvement of the left ventricle. However, the right ventricle should be taken into careful consideration. The early diagnosis may help to increase the event-free survival.
authors.affiliation: Department of Internal Medicine, Texas Tech University Health Science Center, El Paso, TX, U.S.A.
authors.forename: Sarmad
authors.initials: S
authors.lastname: Said
authors.affiliation: Department of Internal Medicine, Texas Tech University Health Science Center, El Paso, TX, U.S.A.
authors.forename: Chad J
authors.initials: CJ
authors.lastname: Cooper
authors.affiliation: Department of Internal Medicine, Texas Tech University Health Science Center, El Paso, TX, U.S.A. ; Division of Cardiology, Texas Tech University Health Science Center, El Paso, TX, U.S.A.
authors.forename: Karla
authors.initials: K
authors.lastname: Quevedo
authors.affiliation: Department of Internal Medicine, Texas Tech University Health Science Center, El Paso, TX, U.S.A.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Rodriguez
authors.affiliation: Department of Internal Medicine, Texas Tech University Health Science Center, El Paso, TX, U.S.A. ; Division of Nephrology, Texas Tech University Health Science Center, El Paso, TX, U.S.A.
authors.forename: German T
authors.initials: GT
authors.lastname: Hernandez
keywords: biventricular noncompaction; dilated cardiomyopathy; trabeculation