Complex Aortic Coarctation, Regurgitant Bicuspid Aortic Valve with VSD and Ventricular Non-Compaction: A Challenging Combination

Abstract

Left ventricular non-compaction is a myocardial disorder characterized by excessive trabeculations and deep recesses that communicate with the ventricular cavity, which is thought to result from a failure of the trabecular regression that occurs during normal embryonic development. It carries a high mortality from heart failure or sudden cardiac death. A 15-year-old female patient was referred to our institution for moderate symptoms of heart failure. Echocardiography and MRI showed a bicuspid aortic valve with severe regurgitation, subaortic VSD, dilated left ventricle and left ventricular non-compaction with a moderately decreased ejection fraction, as well as isthmic coarctation and transverse arch hypoplasia. We elected to perform transaortic VSD closure and aortic valve replacement using a mechanical prosthetic valve on an arrested heart, and to address aortic coarctation and transverse arch hypoplasia using an extra-anatomic ascending-to-descending aorta bypass. Aortic cross-clamping was limited to 41 minutes. The postoperative recovery was rapid and the girl was discharged in NYHA class I with an estimated LVEF of 39%. Although management must be individualized, extra-anatomic bypass is a good single-stage approach for patients with complex coarctation and concomitant cardiovascular or myocardial disorders, reducing ischemic time and offering a better chance of successful weaning from cardiopulmonary bypass.

An infrequent association: Left ventricular non compaction with bicuspid aortic valve and coarctation of the aorta.

“…Thus, the LV had severely enlargement and impairment of ejection fraction. This complex was called by the authors as Laubry-Pezzi syndrome [8].…”

Generation of a Urine-Derived Ips Cell Line from a Patient with a Ventricular Septal Defect and Heart Failure and the Robust Differentiation of These Cells to Cardiomyocytes via Small Molecules

“…VSD is the most frequent form of CHD and its pathogenesis is still unclear due to its genetic complexity and phenotypic variability. Prolonged shunting from left to right through a large VSD may result in Eisenmenger Syndrome and eventually lead to HF [23]. HF is a progressive intractable disease and often occurs due to volume overload secondary to CHDs such as VSD in children, and is thus a major cause of mortality and morbidity worldwide, despite intensive medical and interventional treatments [24].…”

Clinical improvement and no further need of transplant after closing PDA by transcatheter approach in an end stage heart failure patient with hypertrabeculation

“…In these cases, repairing defect resulted in improvement of cardiac function. [5][6] In some asymptomatic cases, or cases with minor symptoms in which PDA and noncompaction cardiomyopathy present together, although the patients did not have clinical heart failure, patients were treated with repair of PDA due to enlargement of left heart chambers seen on echocardiography. [7,8] In our case, advanced heart failure resulted from left-to-right shunt and noncompaction cardiomyopathy.…”

“…In the literature there are documented cases of ventricular septal defect (VSD) or PDA together with noncompaction for which correction of lesions with left-to-right shunts was preferred treatment. [5][6][7][8] Presently described is case of noncompaction cardiomyopathy leading to end-stage heart failure in which heart transplant was avoided due to clinical improvement with repair of PDA.…”