Humans ; Aortopulmonary Septal Defect/surgery* ; Septal Occluder Device

Humans ; Aortopulmonary Septal Defect/surgery* ; Septal Occluder Device

Aortopulmonary window (APW) is a rare cardiac anomaly that was reported to occur in only 43 cases over 33 years at a large-volume cardiac center. It can present as an isolated anomaly or in combination with another cardiac anomaly. The surgical technique for APW has evolved from simple ligation to separation of the 2 great arteries. However, because of the rarity of APW, there is no standard surgical treatment for this disease entity. Herein, we present successful aortic reconstruction using a main pulmonary artery flap after separation of the 2 great arteries in a neonate with isolated APW.
Aortopulmonary Septal Defect ; Arteries ; Humans ; Infant, Newborn ; Ligation ; Pulmonary Artery

Interrupted aortic arch with an aortopulmonary window is a rare congenital entity that is associated with high morbidity and mortality, especially in premature low-birth-weight infants, and the proper timing of surgical correction remains a matter of debate. We present the case of a premature infant weighing 1.6 kg who successfully underwent one stage surgical repair to treat interrupted aortic arch with an aortopulmonary window. The therapeutic management of this patient is described below, and a review of the literature is presented.
Aorta, Thoracic* ; Aortopulmonary Septal Defect ; Humans ; Infant, Low Birth Weight ; Infant, Newborn* ; Infant, Premature ; Mortality

Persistent truncus arteriosus categories associated with different natural histories and various surgical approaches were reported. Although pulmonary overflow and severe heart failure are common, some patients who have hypoplastic pulmonary artery systems may show lesser symptoms of heart failure and remain in relatively stable condition. We experienced a 33-year-old woman with uncorrected type II persistent truncus arteriosus who presented with cyanosis rather than congestive heart failure, and are presenting her images.
Adult* ; Aorta ; Cyanosis ; Female ; Heart Failure ; Humans ; Pulmonary Artery ; Truncus Arteriosus ; Truncus Arteriosus, Persistent*

OBJECTIVE: To analyze the spectrum of prenatally diagnosed congenital heart disease in a Korean population with 22q11.2 deletion syndrome, and to provide guidelines for screening 22q11.2 deletion prenatally. METHODS: This retrospective study evaluated 1,137 consecutive fetuses that had prenatal genetic testing for 22q11.2 deletion because of suspected congenital heart disease between September 2002 and December 2012, at Asan Medical Center, Seoul, Korea. RESULTS: Main cardiovascular diseases in the 53 fetuses with confirmed 22q11.2 deletions were tetralogy of Fallot (n = 24, 45%), interrupted aortic arch (n = 10, 19%), ventricular septal defect (n = 5, 9%), double outlet right ventricle (n = 4, 8%), and coarctation of the aorta (n = 4, 8%). Other cardiac defects were rarely associated with 22q11.2 deletion. One fetus had persistent truncus arteriosus, one had aortic stenosis, and one had hypoplastic right heart syndrome. Two fetuses had normal intracardiac anatomy with an isolated right aortic arch, and one had an isolated bilateral superior vena cava. CONCLUSION: A variety of congenital heart diseases were seen during the prenatal period. Conotruncal cardiac defects except transposition of great arteries were strongly associated with 22q11.2 deletion. When such anomalies are diagnosed by fetal echocardiography, genetic testing for 22q11.2 deletion should be offered. Even if less frequent deletion-related cardiac defects are detected, other related anomalies, such as thymic hypoplasia or aplasia, should be evaluated to rule out a 22q11.2 deletion.
Aorta, Thoracic ; Aortic Coarctation ; Aortic Valve Stenosis ; Cardiovascular Diseases ; Chungcheongnam-do ; DiGeorge Syndrome* ; Double Outlet Right Ventricle ; Echocardiography ; Fetus ; Genetic Testing ; Heart ; Heart Defects, Congenital* ; Heart Diseases ; Heart Septal Defects, Ventricular ; In Situ Hybridization, Fluorescence ; Korea ; Mass Screening ; Retrospective Studies ; Seoul ; Tetralogy of Fallot ; Transposition of Great Vessels ; Truncus Arteriosus, Persistent ; Vena Cava, Superior

Bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Medial abnormalities in the ascending aorta are prevalent in other types of patients with a variety of forms congenital heart disease (CHD), such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, tetralogy of Fallot. These abnormalities encompass a wide age range, and may predispose to dilatation, aneurysm, and rupture that necessitates aortic valve and root surgery. This dilatation can develop in CHD patients without stenotic region. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. The concept of aortic dilatation is shifting a paradigm of aortic dilatation, as so called post stenotic dilatation, to primary intrinsic aortopahy. These aortic dilatation and increased stiffness can induce aortic aneurysm, rupture of the aorta and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow and left ventricular failure. We can recognize this association of aortic pathophysiological abnormality, aortic dilation and aorto-left ventricular interaction as a new clinical entity: "aortopathy".
Aneurysm ; Aorta ; Aortic Aneurysm ; Aortic Aneurysm, Thoracic ; Aortic Coarctation ; Aortic Diseases ; Aortic Valve ; Aortic Valve Insufficiency ; Arteries ; Bicuspid ; Coronary Vessels ; Cysts ; Dilatation ; Elasticity ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Heart Valve Diseases ; Humans ; Hypertrophy, Left Ventricular ; Hypoplastic Left Heart Syndrome ; Polymethacrylic Acids ; Rupture ; Tetralogy of Fallot ; Truncus Arteriosus, Persistent ; Vascular Stiffness ; Ventricular Function, Left

OBJECTIVETo recite early results and long-term outcomes after surgical repair of persistent truncus arteriosus (PTA).

METHODSThe clinic data of 54 patients underwent surgical repair for PTA from January 1999 to December 2009 was analyzed retrospectively. There were 36 male and 18 female patients, with a mean age of (9 ± 10) months (range, 1 to 38 months; median, 5 months). Preoperative mechanical ventilation was required in 5 patients. The surgical procedures were closure of ventricular septal defect and re-establishment of continuity between right ventricle and pulmonary artery. The right ventricular outflow tract (RVOT) was reconstructed by direct anastomosis pulmonary artery to right ventriculotomy with anterior wall patch enlargement (28 cases), or by inserting conduits (26 cases). Valvuloplasty were performed in 4 patients with truncal valves moderate to severe insufficiency and aortoplasty in 3 patients with interrupted aortic arch (IAA).

RESULTSThere were 3 patients (5.6%) died of pulmonary hypertensive crisis in hospital. The mean duration of ventilation was 6.8 days in 5 patients who were intubated before operation, while the others were 3.6 days. Forty-seven (92.2%) patients were followed-up for mean (6.8 ± 2.5) years (from 2.5 to 11.0 years). There were 2 patients with mild to moderate aortic regurgitation. One patient with aortic arch obstruction underwent balloon dilatation 2 years postoperatively. Among those patients who underwent direct anastomoses, 8 (32.0%) patients had pulmonary branch stenosis at 7 months to 1.5 years postoperatively, 12 (48.0%) patients were freedom from surgical reintervention 5.0 to 11.0 years postoperatively. Among those inserting conduits, 7 patients (31.8%) had conduit stenosis at 2.8 to 7.0 years after operation. Reoperations were performed for RVOT in 15 patients and there was no mortality.

CONCLUSIONSIt is difficult to treat the PTA patients with IAA, intra-mural coronary artery or mechanical ventilation support before operation. The technique of direct anastomosis between pulmonary artery and right ventricle offers the potential growth for RVOT, but bilateral pulmonary branch stenosis may be occurred at earlier period of postoperation in some patients.

Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Retrospective Studies ; Treatment Outcome ; Truncus Arteriosus, Persistent ; surgery

Aortopulmonary Septal Defect ; therapy ; Catheterization ; Female ; Humans ; Infant

Child, Preschool ; Echocardiography ; methods ; Humans ; Male ; Truncus Arteriosus, Persistent ; diagnostic imaging ; Ventricular Septum ; diagnostic imaging