Large aortopulmonary collateral artery exclusively supplying the lower lobe of left lung in an infant with common arterial trunk with aortic dominance with confluent pulmonary arteries.

INTRODUCTION: Common arterial trunk with aortic dominance has well-developed bilateral pulmonary arterial arborization without any essential major aortopulmonary collateral arteries (MAPCAs), whereas "solitary" arterial trunk is characterized by collateral arterial supply to all bronchopulmonary segments and absent pulmonary arteries. CASE REPORT: We report a term female neonate with common arterial trunk with aortic dominance with confluent pulmonary arteries with a large MAPCA as the sole blood supply to the lower lobe of the left lung. RESULTS: Initial diagnostic workup missed this MAPCA from the descending thoracic aorta. It was suspected during surgery due to massive left atrial return and confirmed by cardiac catheterization study in the early postoperative period and treated successfully by unifocalization. DISCUSSION: A large essential MAPCA supplying an entire lobe in the common arterial trunk with aortic dominance has not been described. It can present as heart failure and inability to wean off the ventilator in the early postoperative period following intracardiac repair. CONCLUSION: This is a case hitherto undescribed and possibly falling outside the well-entrenched classifications of the common arterial trunk and a large MAPCA could be an addition to the list of lesion modifiers. Our experience with this case underlines the importance of a thorough and open-minded approach to the initial imaging assessment of even well-described conditions.

Double outlet right atrium-A "bridge" across the bridging leaflets.

INTRODUCTION: Mal-alignment between the inter-atrial septum (IAS) and the inter-ventricular septum (IVS) during cardiac embryogenesis results in abnormal atrioventricular (AV) connections ranging from doubleoulet left atrium (DOLA) to double outlet right atrium (DORA)1. CASE REPORT: We report DORA resulting from partial override of the interatrial septum (IAS) across the cleft between the bridging leaflets of the left atrioventricular (AV) valve. RESULTS: Successful surgical management with preservation of the orifice is described, leading to an unusual type of double orifice left AV valve. DISCUSSION: The mal-alignment of the interatrial septum, resulting in it bridging the bridging leaflets, leads to an additional orifice between the RA and the LV and a unique form of right to left shunt. DORA can cause cyanosis depending on the size of the shunt and the compliance of the ventricles. Our patient was acyanotic, possibly due to the small size of the orifice and low LV compliance secondary to hypertrophy of the LV. CONCLUSION: our case adds to the spectrum of atrial septal mal-alignment variant of DORA in the setting of AVSD without atrial and ventricular components, with RA to LV communication occurring via the smaller orifice of a left atrioventricular valve divided by the abnormal insertion of a partially overriding, mal-aligned atrial septum; a mechanism not described previously - the 'bridge' across the bridging leaflets!

Percutaneous Closure of Ventricular Septal Defects in 116 Patients: Experience with different devices.

OBJECTIVES: This study aimed to review the experience with percutaneous closure of ventricular septal defects (VSDs) at the National Heart Center (NHC) in Muscat, Oman. METHODS: This retrospective study was conducted from November 2008 to December 2017. Patients' electronic medical records were reviewed to identify their clinical, imaging and interventional data before and after the procedure and on the last follow-up. RESULTS: A total of 116 patients, the majority of which were female (58%), underwent 118 percutaneous procedures for VSD closure at a median age of 3.54 years (range: 0.25-33 years) and a median weight of 12 kg (range: 3.5-78 kg). The mean diameter of the VSDs as determined by transoesophageal echocardiogram was 5.6 ± 1.9 mm (n = 105). The commonest type of VSD was perimembranous (n = 75, 63.5%). Devices were successfully placed during 111 (94.1%) procedures in 109 (94.0%) patients, with the commonest device being a Amplatzer™ duct occluder I (St. Jude Medical, Little Canada, Minnesota, USA; n = 39, 35.1%). There was no mortality. Early major cardiac complications occurred in six patients (5.5%) with device embolisation being the commonest (n = 4, 3.7%). The median follow-up period was 19 months (range: 1-84 months) in 89 (81.7%) of the patients. One patient (0.9%) required a permanent pacemaker for a complete heart block. CONCLUSION: This study has demonstrated a good rate of VSD closure with low morbidity and no mortality using the percutaneous approach with different devices. Long-term follow-up is needed to specifically evaluate the function of adjacent structures and the long-term effects on conduction systems.

Transcatheter Closure of Ruptured Sinus of Valsalva Aneurysm: Report of two cases.

A ruptured sinus of Valsalva aneurysm (RSVA) is a rare cardiac anomaly. Traditionally, RSVAs were repaired surgically; however, percutaneous transcatheter closure is the current treatment of choice. We report two cases of RSVA which were closed using this approach. The first case was a 45-year-old female who presented to the Royal Hospital, Muscat, Oman, in 2014 with a RSVA in the right ventricle. The second case was a 39-year-old male who was admitted to the Sultan Qaboos University Hospital, Muscat, in 2015 with a large multifenestrated RSVA extending into the right ventricle outflow tract. Each patient underwent transcutaneous cardiac catheterisation using three-dimensional echocardiography. Both interventions were technically successful; however, the second patient required a subsequent surgery due to the continuing presence of a significant shunt. Transcatheter closure of RSVAs is an effective alternative to surgical repair, although large multifenestrated RSVAs should be repaired surgically to ensure complete closure.

Preterm infants with congenital heart disease and bronchopulmonary dysplasia: postoperative course and outcome after cardiac surgery.

OBJECTIVE: Success in treatment of premature infants has resulted in increased numbers of neonates who have bronchopulmonary dysplasia (BPD) and require surgical palliation or repair of congenital heart disease (CHD). We sought to investigate the impact of BPD on children with CHD after heart surgery. METHODS: This was a retrospective, multicenter study of patients who had BPD, defined as being oxygen dependent at 28 days of age with radiographic changes, and CHD and had cardiac surgery (excluding arterial duct ligation) between January 1991 and January 2002. Forty-three infants underwent a total of 52 cardiac operations. The median gestational age at birth was 28 weeks (range: 23-35 weeks), birth weight was 1460 g (range: 431-2500 g), and age at surgery was 2.7 months (range: 1.0-11.6 months). Diagnoses included left-to-right shunts (n = 15), conotruncal abnormalities (n = 13), arch obstruction (n = 6), univentricular hearts (n = 4), semilunar valve obstruction (n = 3), Shone syndrome (n = 1), and cor triatriatum (n = 1). RESULTS: Thirty-day survival was 84% with 6 early and 6 late postoperative deaths. Survival to hospital discharge was 68%. There was 50% mortality for patients with univentricular hearts and severe BPD. The median duration of preoperative ventilation was 76 days (range: 2-244 days) and of postoperative ventilation was 15 days (range: 1-141 days). The median duration of cardiac ICU stay was 7.5 days (range: 1-30 days) and of hospital stay was 115 days (range: 35-475 days). Current pulmonary status includes on room air (n = 14), O2 at home (n = 4), and ventilated at home (n = 4) or in hospital (n = 4), and 5 patients were lost to follow-up. CONCLUSIONS: BPD has significant implications for children who have CHD and undergo cardiac surgery, leading to prolonged ICU and hospital stays, although most survivors are not O2 dependent. Postoperative mortality was highest among patients with univentricular hearts and severe BPD. Optimal timing of surgery and strategies to improve outcome remains to be delineated.