Role of intrapulmonary artery septation in univentricular hearts.

The optimal management of functionally univentricular hearts with a significant unbalanced pulmonary blood flow is unclear. The role of cavopulmonary shunt in the rehabilitation of hypoplastic pulmonary arteries is limited. Intrapulmonary artery septation is a technique, wherein a systemic-to-pulmonary artery shunt provides an additional source of blood flow to rehabilitate the hypoplastic pulmonary artery. We report a case of functionally univentricular heart with hypoplastic left pulmonary artery following a cavopulmonary shunt in whom intrapulmonary artery septation resulted in adequate growth of the left pulmonary artery.

Double aortic arch with atretic proximal left arch.

Aortic arch anomalies are rare and represent about 1% of all congenital cardiac lesions. Double aortic arch with atretic proximal left arch is one of the rare causes of respiratory symptoms in neonates, often missed by preoperative imaging studies. Preoperative identification and differentiating this entity from other arch anomalies is imperative to plan appropriate surgical management.

Aortic uncrossing with concomitant arch augmentation in a rare case of left circumflex aorta with arch hypoplasia.

Retro-esophageal circumflex aortic arch is a rare form of vascular ring. Majority of cases occur in isolation without any intracardiac lesions. Only a few cases of left circumflex aorta have been reported in the literature. We present a case of left circumflex aorta with associated arch hypoplasia and coarctation, ventricular septal defect, who underwent single-stage repair.

Pediatric Cardiothoracic CT Guideline Provided by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group: Part 2. Contemporary Clinical Applications.

The use of pediatric cardiothoracic CT for congenital heart disease (CHD) was traditionally limited to the morphologic evaluation of the extracardiac thoracic vessels, lungs, and airways. Currently, the applications of CT have increased, owing to technological advancements in hardware and software as well as several dose-reduction measures. In the previously published part 1 of the guideline by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group, we reviewed the prerequisite technical knowledge for clinical applications in a user-friendly and vendor-specific manner. Herein, we present the second part of our guideline on contemporary clinical applications of pediatric cardiothoracic CT for CHD based on the consensus of experts from the Asian Society of Cardiovascular Imaging CHD Study Group. This guideline describes up-to-date clinical applications effectively in a systematic fashion.

Primary Arterial Switch Operation for Late Referral of Transposition of the Great Arteries with Intact Ventricular Septum in the Current Era: Do We Still Need a Rapid Two-Stage Operation?

OBJECTIVE: We introduced primary arterial switch operation for the patient with transposition of great arteries and intact ventricular septum (TGA-IVS) who had more than 3.5 mm of posterior left ventricle (LV) wall thickness. METHODS: Between January 2013 and June 2015, a total of 116 patients underwent arterial switch operation. Of the 116 patients, 26 with TGA-IVS underwent primary arterial switch operation at more than 30 days of age. RESULTS: The age and body weight (mean ± SD) at the operation were 120.4 ± 93.8 days and 4.1 ±1.0 kg, respectively. There was no hospital mortality. The thickness of posterior LV wall (preoperation vs postoperation; mm) was 4.04 ± 0.71 versus 5.90 ± 1.3; P < .0001; interval: 11.8 ± 6.5 days. The left atrial pressure (mm Hg; postoperative day 0 vs 3) was 20.0 ± 3.2 versus 10.0 ± 2.0; P < .0001; and the maximum blood lactate level (mmol/dL) was 4.7 ± 1.4 versus 1.4 ± 0.3; P < .0001, which showed significant improvement in the postoperative course. All cases had delayed sternal closure. The patients who belonged to the thin LV posterior wall group (<4 mm [preoperative echo]: n = 13) had significantly longer ventilation time (days; 10.6 ± 4.8 vs 4.8 ± 1.7, P = .0039), and the intensive care unit stay (days) was 14 ± 9.2 versus 7.5 ± 3.5; P = .025, compared with thick LV wall group (≥4.0 mm: n = 13). CONCLUSIONS: The children older than 30 days with TGA-IVS can benefit from primary arterial switch operation with acceptable results under our indication. However, we need further investigation for LV function.

Mitral valve repair for congenital mitral valve disease: impact of the use of a biodegradable annuloplasty ring.

BACKGROUND: This study compares the midterm results of mitral valve repair using the biodegradable ring versus repair with non-ring annuloplasty techniques for congenital mitral valve disease in young children where it was not possible to use standard commercial rings. METHODS: Between February 2006 and November 2011, 68 patients underwent mitral valve repair for congenital mitral valve disease. Thirty-nine (57%) patients underwent concomitant annuloplasty using the biodegradable ring (group A) and 29 (43%) patients using non-ring annuloplasty techniques (group B). The median age of repair was 2 years (range 1 month to 14 years). RESULTS: There were no hospital deaths or major postoperative morbidity. At a median follow-up of 3.8 years (range, 2 months to 5.1 years), overall survival at 5 years was 97% ± 3% for group A and 88% ± 8% for group B (p = 0.29). Freedom from valve failure was 90% ± 7% in group A and 74% ± 9% in group B (p = 0.026). The freedom from reoperation was 100% in group A and 84% ± 9% in group B (p = 0.04). The mean transmitral gradients were 4.3 ± 1.8 mm Hg in group A and 4.2 ±1.7 mm Hg in group B (p = 0.77). CONCLUSIONS: Mitral valve repair using the biodegradable ring for congenital mitral valve disease was superior compared with non-ring annuloplasty repair. Excellent survival benefit, freedom from reoperation, and valve failure without significant elevation of transmitral gradients, suggests its important role in young children with smaller annular sizes where standard commercial rings are not available or recommended.

Inverted left atrial appendage masquerading as a cardiac mass.

An inverted left atrial appendage is a rare phenomenon post cardiac surgery. The lesion presents as an additional mass in the left atrium, which would trigger unnecessary concerns and frequently, a battery of tests. The lesion can be easily diagnosed using echocardiography. We report a case of inverted left atrial appendage in a patient post repair of common arterial trunk. Echocardiographic pictures and features which help to identify this lesion as well as to differentiate it from other possible left atrial mass are described. This article aimed to improve the awareness of sonographers toward this rare but possible post operative lesion.

An accessory mitral valve leaflet causing left ventricular outflow tract obstruction and associated with severe aortic incompetence.

This case report describes a 20-year-old woman with Turner's syndrome who presented with reduced effort tolerance limited by dyspnea. She had previously been on pediatric cardiology follow-up for congenital subvalvular aortic stenosis first diagnosed at age 7. Unfortunately she defaulted after two visits before any intervention could be done. Transthoracic echocardiography demonstrated severe aortic incompetence (AI) with a membrane-like structure in the left ventricular outflow tract (LVOT). The mean pressure gradient across the LVOT on continuous wave Doppler was 41 mmHg. The membranous interventricular septum appeared aneurysmal and it was observed that the "subaortic membrane" had a connection to the anterolateral papillary muscle via a strand of chordal tissue. Further images were captured using two-dimensional and three-dimensional transthoracic and transesophageal echocardiography (iE33, Philips Medical Systems, Andover, MA, USA). After a review of the literature it was concluded that this appeared to be an accessory mitral valve (AMV) leaflet causing LVOT obstruction associated with AI. AMV tissue is a rare congenital malformation causing LVOT obstruction. Because it is so unusual, it may not be immediately recognizable even in a high volume echocardiography laboratory. The clue which helped with the diagnosis was the strand of chordal tissue which connected the mass to the papillary muscle. This anomaly is often associated with LVOT obstruction.

Transcatheter closure of muscular ventricular septal defect using the Amplatzer devices.

TECHNIQUE: From November 1997 to June 2002, percutaneous transcatheter closure of muscular ventricular septal defects was attempted in seven patients. Four patients had single and three had multiple defects. Surgical closure was performed in two patients in an attempt to close a perimembranous defect, leaving behind a large apical muscular defect, which was successfully closed using a device in one patient, whilst the second patient succumbed to septicemia/endocarditis 3 weeks after failure of device implantation. One patient had previous pulmonary artery banding and in another intraoperative placement of two Clamshell devices followed by additional transcatheter closure using Gianturco coils in two different sessions was performed. RESULTS: Transcatheter closure of ventricular septal defect was successful in six patients. Three patients underwent closure of their ventricular septal defects using the Amplatzer Septal Occluder and in the remaining three patients. Amplatzer Muscular Ventricular Septal Defect Occluder was used. The median age was 15 months (range 14 months to 10 years) and the median weight was 7.8 kg (range 7-16 kg). The devices were deployed antegradely in all patients. Immediate complete closure was obtained in three patients while two patients had small and one had a large residual following the procedure. The later was due to another multiple muscular septal defect. Transient heart block occurred in one patient during the procedure and another patient developed heart block on day 3 post-procedure. The latter required temporary pacemaker. During the follow up, one patient who had residual multiple muscular defects underwent a successful redo transcatheter closure using two Amplatzer Muscular Ventricular Septal Occluder devices. CONCLUSIONS: We conclude that transcatheter closure of muscular ventricular septal defect using Amplatzer devices is feasible and effective.

Initial results and medium-term follow-up of stent implantation of patent ductus arteriosus in duct-dependent pulmonary circulation.

OBJECTIVES: This study was designed to assess the safety, efficacy, medium-term outcome, and complications of patent ductus arteriosus (PDA) stenting in duct-dependent pulmonary circulation. BACKGROUND: Patent ductus arteriosus stenting has been proposed as an alternative to surgical shunt on account of postoperative morbidity and complications of surgical shunting. METHODS: Between April 2000 and February 2003, 69 patients with duct-dependent pulmonary circulation underwent cardiac catheterization with the intent of PDA stenting as first palliative procedure. Patients with critical pulmonary stenosis and pulmonary atresia with intact ventricular septum post-radiofrequency valvotomy who had PDA stenting were excluded. Thirteen more patients were excluded because of branch pulmonary artery (PA) stenosis. The follow-up was by clinical examination, echocardiography, and repeat cardiac catheterization at six to nine months following the procedure. RESULTS: Patent ductus arteriosus stenting was successful in 51 patients (91.1%) and failed in 5 patients (8.9%). The mean narrowest PDA diameter was 1.9 +/- 0.6 mm. The mean procedure and fluoroscopy time were 95.7 min and 29.4 min, respectively. In one patient the stent dislodged and migrated to the left femoral artery and another patient developed transient intravascular hemolysis. There was no procedure-related mortality. Three patients (5.9%) died one day to two months after the procedure. At follow-up (3.2 months to 2.4 years), 8 patients developed significant stent stenosis requiring reintervention. Seven patients developed worsening of preexisting branch PA stenosis. The freedom from reintervention was 89% and 55% at 6 months and 1 year, respectively. CONCLUSIONS: Patent ductus arteriosus stenting is an attractive alternative to surgical shunt in a majority of patients with duct-dependent circulation. An absolute contraindication to this technique is the presence of branch pulmonary stenosis.

Transcatheter closure of defects within the oval fossa using the Amplatzer Septal Occluder.

This study reviewed the short-term outcome of transcatheter closure of the defects within the oval fossa using an Amplatzer Septal Occluder. From January 1997 to December 2000, 210 patients with defects within the oval fossa underwent successful transcatheter closure. We reviewed a total of 190 patients with left-to-right shunts, assessing the patients for possible complications and the presence of residual shunts using transthoracic echocardiogram at 24 h, 1 month, 3 months and one year. Their median age was 10 years, with a range from 2 to 64 years, and their median weight was 23.9 kg, with a range from 8.9 to 79 kg. In 5 patients, a patent arterial duct was closed, and in 2 pulmonary balloon valvoplasty performed, at the same sitting. The median size of the Amplatzer device used was 20 mm, with a range from 9 to 36 mm. The median times for the procedure and fluoroscopy were 95 min, with a range from 30 to 210 min, and 18.4 min, with a range from 5 to 144 min, respectively. Mean follow-up was 20.8 +/- 12.4 months. Complete occlusion was obtained in 168 of 190 (88%) patients at 24 h, 128 of 133 (96.2%) at 3 months, and 103 of 104 (99%) at one year. Complications occurred in 4 (2.1%) patients. In one, the device became detached, in the second the device embolized into the right ventricular outflow tract, the lower end of the device straddled in the third, and the final patient had significant bleeding from the site of venupuncture. There were no major complications noted on follow-up. We conclude that transcatheter closure of defects within the oval fossa using the Amplatzer Septal Occluder is safe and effective. Long-term follow-up is required, nonetheless, before it is recommended as a standard procedure.