Ebstein's anomaly with coarctation of the aorta. An unusual association

Abstract

This anomaly has a prevalence of 0.5% among the different congenital heart diseases 1 . Studies show that lesions such as pervious foramen ovale and atrial septal defect (ASD) are almost always present. Other types of associations, such as stenosis or atresia of the pulmonary valve, ventricular septal defect and transposition of the great arteries, are uncommon 2,3 . However, the association of Ebstein's anomaly with coarctation of the aorta is described as extremely rare, mainly when ventriculoarterial concordance is present 4 . This report describes 3 individuals with Ebstein's anomaly and coarctation of the aorta, 2 of them in discordance and the remaining 1 in atrioventricular and ventriculoarterial concordance (AV). Report of the CasesPatients in atrioventricular and ventriculoarterial discordance -Case 1 -A 7-month-old infant with a past medical history of dyspnea on strenuous effort and a heart murmur detected on the 10 th day of life. The physical examination showed an infant in good health, with slight tachydyspnea and without cyanosis. His heart rate (HR) was 160bpm; his systemic blood pressure in the upper limbs was 110/70mmHg. His lungs showed no rales.On physical examination, visible pulsations at the precordium were noted and his heart auscultation showed a split 2 nd heart sound of increased intensity, as well as a moderate systolic murmur in the mitral area, irradiating to the axilla. His liver was felt 4cm below the right costal margin. He had a strong pulse in the upper limbs and no pulse in the lower limbs.His chest X-ray showed a moderately enlarged cardiac silhouette, as well as an increased pulmonary vasculature.The electrocardiogram (ECG) revealed sinus rhythm and a pattern of significant left ventricular hypertrophy. The vectorcardiogram, however, was consistent with biventricular hypertrophy with predominance of the left-sided chamber.The Doppler echocardiography showed AV and ventriculoarterial discordance combined with Ebstein's anomaly of the tricuspid valve, ASD and coarctation of the aorta, with a pressure gradient of 40 mmHg between the ascending and the descending aorta.The angiocardiographic and hemodynamic evaluations confirmed the clinical diagnosis.At that time, due to the lack of clinically significant consequences of the regurgitation of the left AV valve, only the correction of the coarctation of the aorta with terminoterminal anastomosis was performed.During the postoperative period, the infant showed systemic hypertension. This condition was controlled with captopril, which was discontinued 2 months later. Currently, the infant is in good health and, on his physical examination, a slight regurgitation of the left AV valve is noted. The infant remains on low-dose medications for treating congestive heart failure.

Ebstein Anomaly: A Review

“…The most common procedure is the tricuspid valve reconstruction because it is quite feasible; however, it needs to be carried out by a specialist surgeon in a tertiary referral unit. 28 Such a procedure can consist of a valve reconstruction and even placation of the atrialized portion of the right ventricle. Also, the anterior leaflet of the tricuspid valve is incised and afterward repositioned along the tricuspid valve annulus to give rise to a monocuspid right atrioventricular annulus, which is the aim of the whole procedure.…”

Ebstein's anomaly of tricuspid valve with aortic stenosis and coarctation of aorta: Successful single‐stage repair of a rare adult congenital heart disease

“…The associated findings of BAV are occasionally described with varying degrees of stenosis/regurgitation 2 . CoA is one of the most unusual abnormalities associated with Ebstein's anomaly 3 . There are no specific guidelines for symptomatic adults presenting with CoA and Ebstein's anomaly along with bicuspid aortic valvular stenosis 4 .…”

“…2 CoA is one of the most unusual abnormalities associated with Ebstein's anomaly. 3 There are no specific guidelines for symptomatic adults presenting with CoA and Ebstein's anomaly along with bicuspid aortic valvular stenosis. 4 The surgical techniques described separately for each abnormality can be combined in such complex surgeries.…”

Neurological, Extracardiac, and Cardiac Manifestations of Ebstein’s Anomaly Along With its Genetics, Diagnostic Techniques, Treatment Updates, and the Future Ahead

“…Patients must each have a unique treatment plan because this condition is difficult to treat. Tricuspid valve reconstruction is the most popular treatment since it is technically feasible but should always be performed by a cardiac surgeon in an equipped hospital [ 30 ]. Such a surgery can entail softening the atrial region of the right ventricle or possibly valve repair.…”