Isolated Right Ventricular Noncompaction in an Adult Woman with Severe Pulmonary Hypertension Following at the Intensive Care Unit

Abstract

Pulmonary hypertension is defined by a mean pulmonary artery pressure ≥ 25 mmHg at rest, measured during right heart catheterization. Ventricular noncompaction is a genetic cardiomyopathy which mostly effects left ventricle. It is related with deterioration of myocardial embryogenesis and commonly together with other cardiac diseases. Isolated ventricular non-compaction is characterized by modified morphology of myocardial wall, increased trabeculation in ventricular cavity and deep intertrabecular recesses. A 43-year-old woman presented exercise induced dyspnea and atypical chest pain. She has not any medical history prior. On admission, her 12 lead ECG showed complete righ bundle branch block, her blood pressure was 120/80 and pulse rate 80 per minutes. Transthoracic 2D echocardiogram and magnetic resonance imaging showed dilated and hypertrophied right ventricle with non-compaction of the right ventricular apex. The systolic pulmonary arterial pressure was 80 mmHg on the Doppler echocardiography. The coronary angiography revealed normal coronary arteries. The catheterization was showed pulmonary hypertension, right ventricle non-compaction and negative pulmonary vasoreactivity testing. Ventricular noncompaction, especially right ventricular noncompaction, complicated by severe pulmonary hypertension is exceptional. Only a few isolated right ventricular noncompaction has been reported but inclusion of pulmonary hypertesion cases are rare subsets. Diagnosis of pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic ventricular dysfunction secondary to right ventricular noncompaction. Widespread usage of cardiac magnetic resonance imaging, may enhance visual quality and evaluation of ventricular morphology, probably this will provide prevalance increment and clinical outcome improvements. Early diagnosis would bring better results.

Introduction

Pulmonary hypertension is defined by a mean pulmonary artery pressure ≥ 25 mmHg at rest, measured during right heart catheterization. Connective tissue diseases, chronic thrombotic/embolic disease, cardiac and pulmonary shunts and lung disease are associated with pulmonary hypertension. Ventricular noncompaction is a genetic cardiomyopathy which mostly effects left ventricle, rarely involves right ventricle. It is related with deterioration of myocardial embryogenesis and commonly together with other cardiac diseases(1).

Isolated ventricular non-compaction is characterized by modified morphology of myocardial wall, increased trabeculation in ventricular cavity and deep intertrabecular recesses. Two-dimensional echocardiography, cardiac magnetic resonance imaging and right ventriculography can be used for diagnosis. The major clinical manifestations are heart failure, arrhythmias, sudden death and embolic events. In this case, we present right ventricular noncompaction complicated by severe pulmonary hypertension.

Case Report

A 43-year-old woman presented exercise induced dyspnea and atypical chest pain. She has not any medical history prior. There was not any family history of cardiomyopathy or cardiac death. On admission, her 12 lead ECG showed complete righ bundle branch block, her blood pressure was 120/80 and pulse rate 80 per minutes. Transthoracic 2D echocardiogram and magnetic resonance imaging showed dilated and hypertrophied right ventricle with non-compaction (ventricular trabeculations with deep intertrabecular recesses are seen; and color Doppler imaging demonstrates blood flow through these deep recesses in continuity with the ventricular cavity) of the right ventricular apex (Figure 1A, B). The systolic pulmonary arterial pressure was 80 mmHg on the Doppler echocardiography. Secondary causes for pulmonary hypertension like connective tissue diseases, chronic thrombotic/embolic disease, anomalous cardiac and pulmonary shunts and lung disease were excluded. She was received left and right heart catheterization, coronary angiogram and pulmonary vasoreactivity testing with inhalation of iloprost (PGI₂ ). The coronary angiography revealed normal coronary arteries. The catheterization was showed pulmonary hypertension, right ventricle non-compaction and negative pulmonary vasoreactivity testing (Figure 1C). Endothelin receptor antagonist (Bosentan) was prescribed for pulmonary artery hypertension.

Figure 1. . A, B. Transthoracic 2D echocardiography showed dilated and hypertrophied right ventricle with non-compaction (arrow). C. Right ventriculography showing the sponge-like appearance of the noncompacted ventricular Wall and marked retention of the contrast material in the trabecular recesses (arrows).

Discussion

Ventricular noncompaction, especially right ventricular noncompaction, complicated by severe pulmonary hypertension is exceptional. Only a few isolated right ventricular noncompaction has been reported but inclusion of pulmonary hypertesion cases are rare subsets(2). Due to rarity, diagnosis remains extremely difficult.

Mosty congenital heart diseases such as ventricular septal defect, aortic stenosis, coarctation and Ebstein’s anomaly accompany right ventricular noncompaction. As echocardiographic evaluation of right ventricule hardly operator depended, may be some right ventricular noncompaction cases have been underestimated so far. Echocardiography of right ventricule is based on visual appearance. Magnetic resonance imaging may be accurately demonstrate trabeculations and deep recesses also segmental motions(2). Color Doppler may be useful, if there is a remarkable deep perfusion in intertrabecular recesses(3).

Diagnosis of pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic ventricular dysfunction secondary to right ventricular noncompaction. Also, the other underlying mechanism may be associated with embolic events. Widespread usage of cardiac magnetic resonance imaging, may enhance visual quality and evaluation of ventricular morphology, probably this will provide prevalance increment and clinical outcome improvements. Early diagnosis would bring better results.

Informed consent was obtained.

Externally peer-reviewed.

Concept/Design - MY; Analysis/Interpretation - HA; Data Collection - DÖ; Writing - YÖ; Critical Revision - MY; Statistical Analysis - YÖ; Overall Responsibility - MY; Final Approval - All of Authors.

The authors have no conflicts of interest to declare.

The authors declared that this study has received no financial support.

References

1. Awasthy N, Tomar M, Radhakrishnan S. Isolated biventricular noncompaction in an adult with severe pulmonary hypertension: an association reviewed. Images Paediatr Cardiol 2012;14:1-5. [Crossref]
2. Sato Y, Matsumoto N, Matsuo S, Sakai Y, Kunimasa T, Imai S, et al. Right ventricular involvement in a patient with isolated noncompaction of the ventricular myocardium. Cardiovasc Revasc Med 2007;8:275-7. [Crossref]
3. Said S, Cooper CJ, Quevedo K, Rodriguez E, Hernandez GT. Biventricular non-compaction with predominant right ventricular involvement, reduced left ventricular systolic and diastolic function, and pulmonary hypertension in a Hispanic male. Am J Case Rep 2013;14:539-42. [Crossref]

Figure and Tables

Figure 1. . A, B. Transthoracic 2D echocardiography showed dilated and hypertrophied right ventricle with non-compaction (arrow). C. Right ventriculography showing the sponge-like appearance of the noncompacted ventricular Wall and marked retention of the contrast material in the trabecular recesses (arrows).