Left ventricular non-compaction with congenital diaphragmatic hernia causing cardiac dextroposition

Abstract

A 45-year-old man with a 2-month history of breathlessness, fatigue and irregular heart rate was referred to our centre after first-ever cardio-pulmonary decompensation. At presentation he was stable with heart rate of 120/min, blood pressure of 140/90 mmHg, in NYHA class II. 12-lead ECG showed atrial fibrillation, low R-waves voltage and deep S-waves with non specific ST changes in V1-V6. A posterior-anterior chest X-ray showed an enlarged cardiac silhouette, whereas on lateral picture an unusual structure was identified in the thorax cavity. An echocardiogram showed an enlarged, hypertrophied left ventricle (LV) with severe global systolic impairment. A computed tomography revealed an intestinal loop, characterized as the anterior Morgagni type congenital diaphragm hernia, in the anterior mediastinum resulting in the heart dislocation to the right side. Cardiac magnetic resonance revealed a thinned apex coupled with increased trabeculation at the apex, posterior, lateral and anterior wall. The measurements of double-layered LV wall, confirmed the diagnosis of LV non-compaction (LVNC). This case is an illustrate of a constellation of rare anomalies of LVNC, persistent left superior vena cava with a congenital anterior Morgagni-type diaphragm hernia, causing heart dextroposition. On multidisciplinary team meeting the general decision of conservative treatment was made with typical systolic heart failure pharmacotherapy, restoration of sinus rhythm, considering referral to ICD and/or CRT-D, and regular clinical and echocardiographic follow-up of both patient and patient's pedigree. JRCD 2012; 1: 18-23 Case presentationPreviously fit and well 45-year-old man with 2-month history of gradually increasing breathlessness, fatigue and irregular heart rate was referred from the district hospital after cardio-pulmonary decompensation for the exhaustive cardiological work-up. The patient had typical risk factors of cardiovascular diseases such as hypertension, hyperlipidemia and nicotine abuse, otherwise the medical and family history was unremarkable. At presentation he was stable with an irregular heart rate of 120 beats per minute (bpm), arterial blood pressure of 140/90 mm Hg, oxygen saturation of 98%, in New York Heart Association (NYHA) class II. On physical examination he was euvolemic, with normal jugular venous pressure and clear lung fields. Interestingly, heart sounds were more audible at the right side of the chest. Standard 12-lead ECG showed atrial fibrillation, low R-waves voltage and deep S-waves with non specific ST changes in V 1 -V 6 (Figure 1). However, after electrodes were replaced to the right side of the thorax, ECG revealed prominent R waves in precordial leads (Figure 2). A posterior-anterior chest X-ray showed an enlarged cardiac silhouette, whereas on lateral X-ray an unusual structure was identified in the thorax cavity (Figure 3 and 4, arrows). A transthoracic echocardiogram was of poor quality, nevertheless, revealed enlarged and hypertrophied left ventricle (LV) with severe global ...

Isolated left ventricular noncompaction in an asymptomatic althlete (RCD code: III-5A.1.0)

“…LVNC is a rare cardiovascular disease and no specific therapy is available. We previously reported a case of the left ventricular non-compaction in patietn with congenital diaphragmatic hernia causing cardiac dextroposition [12]. In our opinion aspirin as a prevention of thromboembolic complications and ACEI should be considered in asymptomatic patients with diagnosed left ventricular noncompaction and mildly impaired left ventricular function.…”

Overlap cardiomyopathy – coexistence of hypertrophic and restrictive cardiomyopathy phenotypes in one patient (RCD code: III-2A.1)

“…Right heart catherization. Left ventricular pressure tracing with 'dip-and-plateau' or 'square root sign' that represents deep and rapid early decline in ventricular pressure at the onset of diastole, with a rapid rise to plateau in early diastole between LVNC, dextroposition and superior vena cava draining to coronary sinus [10].…”