Prolapsing left atrial myxoma causing severe pulmonary hypertension: dynamic echocardiographic and magnetic resonance imaging

Cardiac myxomas may have different clinical presentations that may expose the patient to sudden death due to obstruction of the left ventricle inflow. The authors report a case of 34 years old male who was diagnosed with left atrial myxoma that presented as mitral valve obstruction with severe pulmonary hypertension. He underwent emergent sternotomy under cardiopulmonary bypass and the myxoma was successfully removed. The postoperative course was uneventful.

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Isolated right ventricular noncompaction in an adult woman with severe pulmonary hypertension following at the intensive care unit

Kosuyolu Heart Journal ◽

10.51645/khj.2021.m148 ◽

2021 ◽

Author(s):

Mustafa Yildiz ◽

Yasemin Ozsahin ◽

Hulya Yilmaz Ak ◽

Dogac Oksen

Keyword(s):

Magnetic Resonance Imaging ◽

Pulmonary Hypertension ◽

Magnetic Resonance ◽

Right Ventricle ◽

Right Ventricular ◽

Heart Catheterization ◽

Severe Pulmonary Hypertension ◽

Resonance Imaging ◽

Atypical Chest Pain ◽

Ventricular Noncompaction

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 (1). 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.

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