Isolated left ventricular apical hypoplasia with myocardial non-compaction: a case report

Abstract

Background Isolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiac abnormality, which might result in severe symptomatic heart failure (HF) with pulmonary hypertension, atrial fibrillation (AF), or malignant ventricular tachycardia in adults. Case summary A 32-years-old man presented with exertional dyspnoea New York Heart Association Class II and persistent AF. Echocardiography and cardiac magnetic resonance showed the presence of (i) spherical remodelling of the left ventricle (LV) with impaired contractile function (three-dimensional ejection fraction, EF 32%); (ii) substitution of apical myocardium by fatty tissue; (iii) abnormal origin of a papillary muscle network; and (iv) an elongated right ventricle, compatible with ILVAH. In addition, non-compacted endomyocardial layer of the LV was observed. Because of a high risk of sudden cardiac death in symptomatic HF patients with reduced EF, an implantable cardioverter-defibrillator was placed which followed by pulmonary vein isolation. After the procedures and restoration of sinus rhythm, the patient demonstrated improvement in HF symptoms and exercise tolerance. This was accompanied by an enhancement of left and right ventricular systolic function by echocardiography. At 6-month, 1, and 2-year follow-up the clinical conditions of the patient and echocardiographic findings remained stable. Discussion A rare combination of ILVAH and left ventricular myocardium non-compaction was observed in this young adult who presented with symptomatic HF and persistent AF. The use of consecutive invasive cardiac procedures leads to restoration of sinus rhythm, the improvement of myocardial contractility and clinical manifestation of HF.

Ventricular Noncompaction With Left Ventricular Thrombus: A Case Report

“…A recent literature review suggests that aspirin alone is not enough for stroke prevention in these patients [ 2 ]. Two case reports suggest that direct oral anticoagulants (DOACS) may be appropriate as well [ 12 - 13 ], especially given the findings of complete resolution of LV thrombus in non-NCC patients with equal efficacy using DOAC therapy [ 11 , 14 ]. Other studies argue that warfarin is superior to DOACs in preventing embolism in patients with evidence of LV thrombus [ 15 ].…”

Isolated left ventricular apical hypoplasia: case report

“… 1 Case reports have increased through years with a wide clinical spectrum ranging from asymptomatic children 2 to severely symptomatic adults with associated heart failure (HF), pulmonary hypertension (PH), and malignant tachyarrhythmias. 3 , 4 Hence, we present the first case of ILVAH reported in Peru and Latin America of a young adult who developed some of the aforementioned complications.…”

A proposed strategy for anticoagulation therapy in noncompaction cardiomyopathy

“…57,58 Similarly, several case reports have demonstrated that DOACs could be at least as effective and safe as warfarin in preventing cardioembolic events in NCCM patients. 59,60 Skidan et al reported a case of a 32year-old male with NCCM and isolated LV apical hypoplasia admitted to the hospital for radiofrequency catheter ablation of persistent AF. 60 Despite the high thromboembolic risk due to the concomitant presence of AF and biventricular systolic dysfunction, no thromboembolic events occurred during 2 years of follow-up in NCCM patients receiving long-term oral anticoagulation therapy with Rivaroxaban.…”

“… 57 , 58 Similarly, several case reports have demonstrated that DOACs could be at least as effective and safe as warfarin in preventing cardioembolic events in NCCM patients. 59 , 60 Skidan et al . reported a case of a 32‐year‐old male with NCCM and isolated LV apical hypoplasia admitted to the hospital for radiofrequency catheter ablation of persistent AF.…”

“…59,60 Skidan et al reported a case of a 32year-old male with NCCM and isolated LV apical hypoplasia admitted to the hospital for radiofrequency catheter ablation of persistent AF. 60 Despite the high thromboembolic risk due to the concomitant presence of AF and biventricular systolic dysfunction, no thromboembolic events occurred during 2 years of follow-up in NCCM patients receiving long-term oral anticoagulation therapy with Rivaroxaban. Similarly, Li et al described the history of a 54-year-old man with hypertension, diabetes, and end-stage renal disease presented with 1 day palpitations and lightheadedness following a dialysis session.…”