Does the right ventricle size influence the left ventricle size and function in children with Ebstein anomaly?

PURPOSE: Patients with Ebstein anomaly (EA) have a variety of clinical manifestation. The assessment of structural and geometric characteristics of the heart is important for optimal management. METHODS: We retrospectively analyzed echocardiography database from 2009 to 2020. We evaluate patients in two groups: patients with EA were in Group 1 and children without cardiovascular pathology were in Group 2. All children in both groups underwent echocardiography according to American Society of Echocardiography recommendations. The shape of the heart chambers and their function were studied in both groups. RESULTS: There were 153 in Group 1 and 2000 children without cardiovascular disease in Group 2. It was shown that in children with EA, the shape of the ventricle became less spherical, which was accompanied by a decrease in myocardial mass, and the ejection fraction was reduced 34% of patients. The functional volume (non-atrialized part) of the right ventricle in patients with EA was reduced, and its contractility was preserved in 62% of cases. Preservation of the contractile properties of the right ventricle in most cases was associated with higher systolic pressure in its cavity. CONCLUSION: TAPSE, TESV, and the velocity of the annulus fibrous ring movement according to tissue dopplerography in patients with EA do not allow us to assess the contractility of the right ventricle. The myocardial performance index (MPI) characterizes a decrease in the functional volume of the right ventricle.

Surgical Management of a Giant Left Ventricular Fibroma in a Newborn.

This clinical case report describes surgical management of a giant left ventricular fibroma and postoperative venoarterial extracorporeal membrane oxygenation support in a newborn male. Transthoracic echocardiography performed at 9-month follow-up showed an ejection fraction of 33% and a cardiac index of 4.5 liters per minute per square meter.

Surgery for aortic recoarctation in children less than 10 years old: A single-center experience in Siberia, Russia.

BACKGROUND: Persistence or recurrence of stenosis is a complication of initial coarctation repair. This study aims to report short-term outcomes of surgical management of recurrent coarctation and initial repair analysis. METHODS: We retrospectively reviewed our experience with 51 patients undergoing recoarctation surgical repair between 2008 and 2019 using antegrade cerebral perfusion (ACP) technique. RESULTS: Surgical correction included prosthetic patch aortoplasty in 23 (45%), resection with wide end-to-end anastomosis in 15 (29%), and a tube interposition graft in 13 (25%) patients. The median age at initial correction and reintervention was 12 months and 9 years. The median interval from primary repair to reintervention was 60 months. Initial repair analysis revealed 33% of patients had initial correction in the neonatal period, 72.5% of patients were done via a left thoracotomy approach and 63% of patients had end-to-end anastomosis at initial surgery. CONCLUSION: Our study demonstrates that surgical repair of recurrent coarctation of the aorta using ACP technique can be performed safely and with excellent results.

Surgical management of a giant congenital left ventricular aneurysm in a 2-month-old infant.

A congenital left ventricular aneurysm is very rare. Clinical presentation varies from absence of symptoms to ventricular arrhythmias, heart failure or even sudden death. The optimal management is controversial and the surgical technique is not defined. A left ventricular aneurysm was diagnosed on prenatal echocardiography at 33 weeks gestation. After birth, initial transthoracic echocardiography confirmed the diagnosis. Two months later, the infant was taken to surgery for aneurysm repair using the Dor procedure with cardiopulmonary bypass. This technique eliminates the need for external prosthetic materials and produces a more physiologic left ventricular geometry. Transthoracic echocardiography performed at 6-month follow-up showed an ejection fraction of 66%.

Surgical Management of Right Aortic Arch Obstruction Associated With Rare Form of Vascular Ring.

This clinical case demonstrated surgical management for a rare case of vascular ring associated with an elongated and kinked aortic arch and a right descending aorta in a ten-year-old male using an extra-anatomic bypass grafting method and dividing the vascular ring. Computer tomography performed at six-month follow-up showed a favorable surgical outcome.