Transcatheter Fontan completion: Creation of an extracardiac Fontan.

Patients with functionally univentricular hearts are usually palliated surgically. There have been several reports of successful attempts to complete the Fontan procedure without surgery. The pathways created at the time of the preconditioning were largely reminiscent of the lateral tunnel Fontan. However, this approach is still confidentially limited to a small number of centers. In 2013, we designed a circuit that mimics the actual surgical technique of extracardiac total cavopulmonary connection to allow for transcatheter completion in an animal study. A polytetrafluoroethylene conduit was connected between the pulmonary artery and the inferior vena cava (IVC). The superior anastomosis was occluded to avoid flow between IVC and superior vena cava (SVC). The conduit was connected to the right atrium (RA) and a large fenestration was created to allow free flow from the IVC to the RA. Extrapolating our approach, a center reported the successful transcatheter completion of an extracardiac Fontan in a 6-year-old child. However, this technique is not directly transposable to our population of patients who require preconditioning in infancy. We report here an innovative extension of this technique that may allow preparing patients in infancy, ideally at the time of the Glenn in the future, to receive an extracardiac Fontan at 2 years/11 kg without additional surgery.

Surgical strategies and novel alternatives for the closure of ventricular septal defects.

A variety of therapies are available to close ventricular septal defects (VSDs). These include surgical closure on bypass, percutaneous device closure, as well as perventricular hybrid closure. Due to the incidence of heart block (1-5%) associated with percutaneous device closure of perimembranous VSDs, surgical closure presently remains the gold standard and preferred therapy for these defects. Therapeutic options are more varied for muscular VSDs. Beyond infancy, transcatheter closure offers excellent results with low morbidity and mortality, without the need for cardiopulmonary bypass. Infants however have a higher incidence of adverse events using a percutaneous approach. Large mid-muscular VSDs in infants can be treated successfully using a hybrid approach, surgical closure on bypass or a percutaneous approach. However, VSDs located apically or anteriorly are difficult to identify surgically and for these infants, perventricular hybrid closure should be considered as the preferred therapeutic modality. However, some VSD's also can be closed percutaneously.

Bilateral diaphragmatic palsy after congenital heart surgery: management options.

Diaphragmatic paralysis following phrenic nerve injury is a major complication following congenital cardiac surgery. In contrast to unilateral paralysis, patients with bilateral diaphragmatic paralysis present a higher risk group, require different management methods, and have poorer prognosis. We retrospectively analysed seven patients who had bilateral diaphragmatic paralysis following congenital heart surgery during the period from July, 2006 to July, 2014. Considerations were given to the time to diagnosis of diaphragm paralysis, total ventilator days, interval after plication, and lengths of ICU and hospital stays. The incidence of bilateral diaphragmatic paralysis was 0.68% with a median age of 2 months (0.6-12 months). There was one neonate and six infants with a median weight of 4 kg (3-7 kg); five patients underwent unilateral plication of the paradoxical diaphragm following recovery of the other side, whereas the remaining two patients who did not demonstrate a paradoxical movement were successfully weaned from the ventilator following recovery of function in one of the diaphragms. The median ventilation time for the whole group was 48 days (20-90 days). The median length of ICU stay was 46 days (24-110 days), and the median length of hospital stay was 50 days (30-116 days). None of the patients required tracheostomy for respiratory support and there were no mortalities, although all the patients except one developed ventilator-associated pneumonia. The outcome of different management options for bilateral diaphragmatic paralysis following surgery for CHD is discussed.

High incidence of abdominal venovenous collaterals after Kawashima operation.

INTRODUCTION: Systemic venous collaterals have been found at different stages of single-ventricle palliation, specifically after Kawashima operation. In this study, we present the incidence, clinical features, associated risk factors, and management of such venovenous collaterals (VVCs) after Kawashima procedure. MATERIALS AND METHODS: A retrospective review of all the patients who underwent Kawashima procedure prior to December 2011 at Hamad General Hospital, Qatar, was performed. Data were collected and reviewed from medical records. RESULTS: The study group involved a total of six patients with Kawashima procedure. The median age of patients was 112.7 months (range 46-336 months), and median age at the time of Kawashima operation was 31.7 months (range 15-187 months). Mean systemic arterial oxygen saturation (Spo 2) after Kawashima operation was 94.5% ± 1.5%. In five patients, during a median follow-up of 53.4 months (range 16.9-147.9 months), the Spo 2 declined to a mean of 78.8% ± 8.2%. A total of 16 venous collaterals were observed in the study group, and majority (70%) of these collaterals were subdiaphragmatic. CONCLUSION: In our study, abdominal VVCs were observed in 100% of the patients who were followed after Kawashima operation. We therefore recommend that thorough evaluation for such VVCs should be part of the ongoing evaluation of patients after Kawashima operation, especially in those with low Spo 2. We also recommend early surgical rerouting of hepatic veins to pulmonary arteries in all post-Kawashima patients.

Urgent hepatic to azygous vein connection for thrombosis of hepatic vein to pulmonary artery conduit.

We report on an emergent use of hepatic-to-azygous vein connection using a 10-mm polytetrafluoroethylene graft in a 6-year-old girl who developed acute thrombosis of an extracardiac graft conduit from the hepatic veins to the pulmonary arteries. She had left atrial isomerism, interrupted inferior vena cava with azygous continuation, and had undergone surgical creation of bilateral superior cavopulmonary connections 5 years earlier. The emergency procedure to create hepatic-to-azygous connection resulted in successful salvage of the patient who was subsequently able to maintain stable oxygen saturations and experienced rapid regression of hepatic congestion and pleural effusions. Six months following the procedure, oxygen saturation was above 90%. Repeated echocardiography and computed tomography angiography showed patency of the hepatic-to-azygous connection.

Further insights into the syndrome of prolapsing non-coronary aortic cusp and ventricular septal defect.

Ventricular septal defect (VSD) with prolapse of the right coronary cusp and aortic regurgitation can be managed surgically with the anatomical correction technique. However when the VSD is located underneath the non coronary cusp surgical management differs due to anatomical constraints and secondary pathological changes seen in the non coronary cusp. It is therefore important that the location of the VSD and the morphology of prolapsing cusp be characterised preoperatively in order to plan appropriate surgical repair. We present a case study in which we discuss the salient differences in the surgical management of the prolapsing right and the prolapsing non coronary cusps.

The Boy from Fez: A tale of five charities, and the global community, joining hands.

A case study in global cooperation between multiple charities and cardiac centers helped to save a 8-year-old Moroccan boy suffering a case of Laubry-Pezzi syndrome with severe aortic regurgitation and ventricular septal defect. His recuperation was aided by the support of communities in both Morocco and Qatar.

Anterior stabilisation of sacroilliac joint for complex pelvic injuries.

Sacroilliac joint diasthesis from high energy trauma is always complicated with chronic pain and long term morbidity. Open anterior stabilisation with plate allow direct reduction and stabilisation with biomechanically advantages. Here we report on four cases of pelvic injury with sacroiliac joint disruption treated with anterior plate stabilisation through a surgical approach similar to that used for anterior ring fractures.

Neoaortic bicuspid valve in arterial switch operation: mid-term follow-up.

BACKGROUND: We sought to identify the prevalence of bicuspid pulmonary valve among patients with transposition of the great arteries undergoing the arterial switch operation and evaluate functional integrity of that valve in the neoaortic position. METHODS: Between October 1985 and December 2001, 391 patients had an arterial switch operation for transposition and its variants. Perioperative information and follow-up data were available for 342 patients. The serial echocardiograms of patients with bicuspid pulmonary valve were reviewed. The neoaortic valve was serially assessed, focusing on aortic insufficiency, annulus diameter, and pressure gradients. RESULTS: Twenty-four patients (7%) had a bicuspid pulmonary valve. Age at operation was 5 days to 12 years. Two patients were lost to follow up, and 22 patients had mean follow-up of 5.3 years (range, 2 months to 13 years), of which 21 patients were alive and 1 died late. At least two postoperative echocardiogram reports were available on 19 patients. Seven patients had no neoaortic regurgitation, and 10 had trivial regurgitation. Severe aortic regurgitation developed in 1 patient with endocarditis and in another with repair of Taussig-Bing anomaly. Neoaortic valve size indexed to body surface area showed an increase in annular diameter over time proportional to somatic growth. No significant valve stenosis developed. CONCLUSIONS: Encountering a bicuspid pulmonary valve at the time of an arterial switch operation is not uncommon. The integrity of a bicuspid pulmonary valve in the neoaortic position is maintained at a mean follow-up of 5.3 years. We believe that the presence of a bicuspid pulmonary valve is not a contraindication to an arterial switch operation.

Fontan completion without surgery.

OBJECTIVE: There are several modifications introduced in the preparation for a subsequent non-surgical transcatheter completion of the Fontan procedure. We report our experience with one type of the modification and the short-term results following its implementation. METHODS: During bidirectional cavopulmonary connection (BCPC) an intra-atrial lateral tunnel is additionally created, as intended for a Fontan procedure but fenestrated with a 10-14 mm aperture. The cardiac end of the superior vena cava (SVC) is then patched to maintain the physiology of BCPC. During the interventional transcatheter completion procedure, the SVC patch is perforated using radio-frequency (RF) energy, balloon-dilated, and stented as well. The aperture is closed with a device when required. Paired t-test was used to compare data before and after the Fontan completion. RESULTS: From June 2003 to February 2006, 16 patients (9 boys and 7 girls, mean age 12 months) underwent the surgical procedure described. The mean bypass time was 137 min and the mean ischemic time was 77 min. There were no operative deaths. One patient with bilateral SVC required a take down due to recurrent effusions. Ten months later, nine patients underwent completion (mean age 20 months, mean weight 10.6 kg). The stents were dilated to a mean diameter of 14.4mm. All except one aperture was closed with a device. The mean fluoroscopy time was 41 min. Oxygen saturation increased from 85 to 94% (p=0.001). Pulmonary artery pressures remained normal (16 mmHg before and 19 mmHg after, p=0.12). No patients required mechanical ventilation and none developed pleural effusions or arrhythmias. All were discharged from hospital within 6 days of the Fontan completion. Twenty-two months after Fontan, all were well. Echocardiography revealed no gradients across the stents. Two patients had minor leaks across the aperture. One underwent further stent dilatation a year later. CONCLUSIONS: Fontan completion without surgery is suitable in patients with single ventricles with lower mortality and morbidity, avoids multiple surgical interventions while maintaining the staged approach and allows for successive dilatation of the Fontan pathway to accommodate for growth.

Repair of atrioventricular valve regurgitation in the modified Fontan operation.

OBJECTIVE: To evaluate the prevalence, impact and outcome of repair of atrioventricular valve regurgitation (AVVR) in Fontan patients. METHODS: We retrospectively reviewed 340 Fontan patients from 1986 to 2001. Twelve patients with valve closure or replacements were excluded. AVVR was graded by transthoracic echocardiography. Patients were divided into group O (no AVVR), group A (1+ to 2+) and group B (3+ to 4+). RESULTS: AVVR was present in 129 (39.3%) patients. Repair was carried out in 37 (11.3%) with no difference in mortality as compared to no repair (18.9 vs 10.9%, P =0.16). Mean follow-up was 44 months (1-197 months) with 14 patients lost to follow-up. No valve repair was carried out in group O (192 patients) and no clinical change in AVVR was observed (pre-op 0.00, post-op 0.78). In group A (85 patients) 6 patients had valve repair without significant change in the degree of AVVR after valve repair (pre-op 1.8, post-op 2.2, P = 0.18). In group B (44 patients) 31 had valve repair and a significant reduction was observed (pre-op 3.28, post-op 2.44, P < 0.001). A similar improvement was also observed when the valve was not repaired (pre-op 3.25, post-op 2.44, P = 0.003). Survival at 10 years was comparable (83% repaired, 89% not repaired, P = 0.165). There was no difference in the incidence of long-term complications (26% repaired, 29.7% no repair, P = 0.64). CONCLUSIONS: Trivial to mild AVVR remains stable and their repair during the Fontan operation provides no additional benefits. Valve repair in patients with moderate to severe AVVR improved the regurgitation with comparable operative mortality and long-term outcome; however, similar benefits could be achieved without repair of the atrioventricular valve. We should not deny patients with similar AVVR the Fontan operation.