Emre Belli

The “Physio-Ring”: an advanced concept in mitral valve annuloplasty

BACKGROUND A new annuloplasty ring has been developed with the aim of adding flexibility to the remodeling annuloplasty concept. Here we report its clinical use with special emphasis on segmental valve analysis and valve sizing. METHODS From October 1992 through June 1994, 137 patients aged 4 to 76 years (mean age, 49.1 years) were operated on. The main causes of mitral valve insufficiency were degenerative, 90; bacterial endocarditis, 15; and rheumatic, 13. The indication for operation was based on the severity of the mitral valve insufficiency (90 patients were in grade III or IV) rather than on functional class (60 patients were in class III or IV). At echocardiography 6 patients had normal leaflet motion (type I), 119 leaflet prolapse (type II), and 12 restricted leaflet motion (type III). Surgical repair was carried out using Carpentier techniques of valve reconstruction. In 3 patients, inadequate ring sizing was responsible for systolic anterior motion of the anterior leaflet diagnosed by intraoperative echo. The valve was replaced in 2 patients. There were three hospital deaths, no late deaths, one reoperation for recurrent mitral valve insufficiency due to chordal rupture 1 month after repair, one reoperation for atrial thrombus formation 5 months after repair, one anticoagulant-related hemorrhage, and one thromboembolic episode. RESULTS Mid-term follow-up between 6 and 18 months was available in 94 patients. Echocardiography showed trivial or no regurgitation in 93.2% of the patients and minimal regurgitation in 6.8%. The average transmitral diastolic gradient was 3.55 +/- 1.93 mm Hg. Left ventricular end-systolic diameter and volume decreased postoperatively, demonstrating an improved left ventricular function. CONCLUSIONS This preliminary experience has provided promising results and allowed us to define the indications of the Physio-Ring versus the classic ring. It has also shown that valve sizing and proper ring selection are of primary importance.

Coronary Events After Arterial Switch Operation for Transposition of the Great Arteries

Background—Transfer of the coronary arteries is a crucial step during the arterial switch operation (ASO) for transposition of the great arteries. This retrospective study aims to assess the incidence and risk factors of coronary events after ASO and sensitivity of noninvasive tests in the diagnosis of the coronary obstruction. Methods and Results—Between 1982 and 2001, 1304 newborn and infants had an ASO and the 1 198 hospital survivors had a 59-month mean follow-up. Coronary events occurred in 94 patients (7.2%; 95% CI, 6 to 9). Survival without coronary events were 92.7, 91, and 88.2% at 1, 10, and 15 years, respectively. The incidence was bimodal: high early and slow later. Multivariate analysis showed correlation with type B or C coronary pattern and major operative events (P <0.0001 and P =0.0024). In a subset of 324 patients who underwent a coronary artery angiography, lesions were observed in 22 patients (6.8%; 95% CI, 5 to 10). Multivariate analysis showed correlation with only type B or C coronary pattern (OR=20.8, P =0.0002). All of these patients had electrocardiogram and echocardiogram, 174 patients also had a treadmill test, and 115 patients had a myocardial scintigraphy. The association of these tests had the highest diagnosis sensitivity, 75%. Conclusion—After ASO, coronary events are not rare, occurring most often early and are an important cause of death. Coronary repair can be needed lately. Noninvasive tests are not sensitive enough to detect significant delayed coronary artery stenosis and coronary artery angiography should be performed.

Surgical management of progressive pulmonary venous obstruction after repair of total anomalous pulmonary venous connection

BACKGROUND The occurrence of a progressive pulmonary venous obstruction after the repair of the total anomalous pulmonary venous connection is a severe complication. OBJECTIVES The objectives of this study were to retrospectively review the patients with this condition and to report our experience with a new surgical technique with a sutureless in situ pericardium repair. METHODS Of 178 patients who underwent correction of total anomalous pulmonary venous connection, 16 patients (9%) experienced the development of a progressive pulmonary venous obstruction in a median interval of 4 months (5 weeks-12 years). Three patients had isolated anastomotic stenosis, 4 patients had isolated pulmonary venous ostial stenosis, and 9 patients had both. Pulmonary venous obstruction was bilateral in 7 patients. The surgical procedures used at reoperation included 8 patch enlargements, 5 ostial endarterectomies, 1 intraoperative stenting, and 7 sutureless in situ pericardium repairs. RESULTS There were 4 deaths after reoperation (4 of 15 patients; 27%). The only significant mortality risk factor was the bilateral location of the pulmonary venous obstruction (P =.045). In patients with isolated anastomotic stenosis or with only 1 pulmonary venous ostial stenosis (n = 5), there was no death, except the patient presenting with a single ventricle. In patients with 2 or more pulmonary venous ostial stenoses (n = 10), there were 3 deaths; 5 of the 7 survivors were successfully treated with the in situ pericardial technique, with normalized pulmonary artery pressure at a mean follow-up of 26 months. CONCLUSION Progressive pulmonary venous stenosis after repair of total anomalous pulmonary venous connection remains a severe complication when bilateral. The sutureless in situ pericardial repair offers a satisfactory solution, particularly on the right side.

Results of the double switch operation for congenitally corrected transposition of the great arteries

BACKGROUND Congenitally corrected TGA (CC-TGA) is characterized by discordant atrioventricular and ventriculo arterial connections. In absence of right ventricular outflow tract obstruction (RVOTO), repair by atrial and arterial switches remains a challenging procedure for which long term follow-up is uncertain. METHODS From 1995 to 2007, 20 patients (median age: 26 months) with CC-TGA had double switch procedure. Segmental anatomy was {SLL} in all patients, dextrocardia in two patients, mesocardia in two patients. Ventricular septal defect was present in 17 patients, aortic coarctation in 2 patients and interrupted aortic arch (IAoA) in 1 patient. Five patients had tricuspid valve regurgitation. Six patients had AV blocks, 4 patients had pacemaker implantation prior to repair. Pulmonary artery banding was performed in 17 patients, for congestive heart failure (14 patients) or left ventricular retraining (3 patients). Three patients, including one patient with IAoA had primary repair. After LV retraining, repair was performed when indexed LV mass to LV volume ratio was above 1.5. A median follow-up of 60 months was achieved in all. RESULTS There were no deaths. Postoperative pacemaker implantation was required in four patients. Reoperation for Senning obstruction was necessary in one patient, and pacemaker battery replacement in another patient. One patient had mild neoaortic insufficiency, two had mild tricuspid regurgitation and two had mild mitral regurgitation. All were in NYHA I-II. Actuarial survival at 10 years was 100% and freedom from reoperation at 5 and 10 years were 93% and 77.4%, respectively. CONCLUSION Double switch for CC-TGA without RVOTO can be performed with no mortality and low morbidity. Since these results seem to last for several years, it should be considered as the optimal procedure.

Surgical treatment of subaortic stenosis: A seventeen-year experience

OBJECTIVE The aim of the study was to analyze the long-term results of subaortic stenosis relief and the risk factors associated with recurrence and reoperation. METHODS One hundred sixty patients with subaortic stenosis underwent biventricular repair. Before the operation the mean left ventricle-aorta gradient was 80 +/- 35 mm Hg, 57 patients had aortic regurgitation, and 34 were in New York Heart Association functional class III or IV. Median age at repair was 10 years. For discrete subaortic stenosis (n = 120), 39 patients underwent isolated membranectomy, 67 underwent membranectomy with associated septal myotomy, and 14 underwent septal myectomy. Tunnel subaortic stenosis (n = 34) was treated by myotomy in 10 cases, myectomy in 12, septoplasty in 7, Konno procedure in 3, and apical conduit in 2. Aortic valve replacement was performed in 6 cases, mitral valve replacement in 2 cases, and mitral valvuloplasty in 4 cases. RESULTS There were 5 early (3.1%) and 4 late (4.4%) deaths. Within 3.6 +/- 3.3 years a recurrent gradient greater than 30 mm Hg was found in 42 patients (27%), 20 of whom had 26 reoperations. According to multivariable Cox regression analysis survival was influenced by hypoplastic aortic anulus (P =.01) and mitral stenosis (P =.048); recurrence and reoperation were influenced by coarctation and immediate postoperative left ventricular outflow tract gradients. At a median follow-up of 13.3 years, mean left ventricle-aorta gradient was 20 +/- 13 mm Hg. Relief of the subaortic stenosis improved the degree of aortic regurgitation in 86% of patients with preoperative aortic regurgitation. Actuarial survival and freedom from reoperation rates at 15 years were 94% +/- 1.3% and 85% +/- 6%, respectively. CONCLUSION Although surgical treatment provides good results, recurrence and reoperation are significantly influenced by previous coarctation repair and by the quality of initial relief of subaortic stenosis.

Potts Shunt in Children With Idiopathic Pulmonary Arterial Hypertension: Long-Term Results

BACKGROUND Idiopathic pulmonary arterial hypertension (IPAH) remains a progressive fatal disease. Palliative Potts shunt has been proposed in children displaying suprasystemic IPAH. METHODS A retrospective multicenter study was performed to evaluate Potts shunt in pediatric IPAH. RESULTS Between 2003 and 2010, 8 children with suprasystemic IPAH and in World Health Organization functional class IV despite medical pulmonary arterial hypertension therapy underwent Potts shunt. Age at IPAH diagnosis ranged from 4 to 180 months (median age, 64 months). Surgical procedure was performed in a mean delay of 41.9±54.3 months (range, 4 to 167 months; median delay, 20 months) after IPAH diagnosis. Mean size of the Potts shunt was 9.25±3.30 mm. Two patients, whose medical pulmonary arterial hypertension therapy had been interrupted just after surgery, died at postoperative days 11 and 13 of acute pulmonary hypertensive crisis. After a mean follow-up of 63.7±16.1 months, the 6 children who were discharged from the hospital were alive. Functional status improved markedly in the 6 survivors, with a World Health Organization functional class I (n=4) or II (n=2) at last follow-up, consistent with significant improvement of 6-minute-walk distance (302±95 m [51%±20% of theoretical values] versus 456±91 m [68%±10% of theoretical values]; p=0.038) and decrease of brain natriuretic peptide levels (608±109 pg/mL versus 76±45 pg/mL; p=0.035). No Potts shunt was found to be restrictive at last echocardiography. CONCLUSIONS Palliative Potts shunt constitutes a new alternative to lung transplantation in severely ill children with suprasystemic IPAH, carrying a prolonged survival and persistent improvement in functional capacities.

Systemic pulmonary shunts in neonates: early clinical outcome and choice of surgical approach.

BACKGROUND Systemic pulmonary shunt remains a major strategy for the palliation of cyanotic lesions in neonates despite the associated morbidity and mortality. METHODS Between March 1993 and December 1998, 79 systemic pulmonary shunts were performed in 75 neonates with cyanosis and severely reduced pulmonary blood flow. The mean age was 11.5 days and the mean weight, 3 kg. All neonates were dependent on duct flow and prostaglandin E1 infusion for adequate oxygenation. RESULTS The systemic pulmonary shunt was performed through a right thoracotomy in 36 patients, left thoracotomy in 6, and median sternotomy in 33 patients. The 30-day mortality was 3 patients (4%). Univariate and logistic regression analyses revealed a weight less than 2 kg (p = 0.039) and preoperative mechanical ventilation (p = 0.008), to be predictors of early mortality, whereas pulmonary hypoplasia (p = 0.55), diagnostic group (p = 0.79), shunt size (p = 0.2), and surgical approach (p = 0.5) were not. There were seven episodes of shunt-related complications that required early intervention. CONCLUSIONS Systemic pulmonary shunt remains an effective palliative measure in cyanotic neonates despite specific complications. Both low weight and preoperative ventilation represent significant risk factors for early mortality.

Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle

OBJECTIVES Increased afterload and multilevel LV obstruction is constant. We assumed that restoration of normal loading conditions by relief of LV obstructions promotes its growth, provided that part of the cardiac output was preoperatively supported by the LV, whatever the echocardiographic indexes. BACKGROUND Whether to perform uni- or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal LV (hypoplastic left heart syndrome classes II & III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision. METHODS Twenty ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multilevel LV obstruction. Preoperative echocardiographic assessment showed: mean EDLW of 12.4 +/- 3.03 ml/m2 and mean Rhodes score of -1.73 +/-0.8. Surgery consisted in relief of LV outflow tract obstruction by coarctation repair in all associated to aortic commissurotomy in one and ASD closure in 2. RESULTS There were 3 early and 2 late deaths. Failure of biventricular repair and LV growth was obvious in patients with severe anatomic mitral stenosis. The other demonstrated growth of the left heart. At hospital discharge the EDLVV was 19.4+/-3.12 ml/m2 (p = 0.0001) and the Rhodes score was -0.38+/-1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were 72.5% and 46%, respectively. CONCLUSIONS Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal LV provided that all anatomical causes of LV obstruction can be relieved. Secondary growth of the left heart then occurs; however, the reoperation rate is high.

Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients

OBJECTIVES Palliative Potts shunt has been proposed in children with suprasystemic pulmonary arterial hypertension (PAH). METHODS A retrospective multicentre study was performed to assess short- and long-term outcomes after Potts shunt. RESULTS From 2003 to 2014, 24 children underwent a Potts shunt [19 surgical, median age: 7.7 years (1.5-17 years), median weight: 19.5 kg (10.2-47 kg) and 5 transcatheter, median age: 8.1 years (2.3-9.7 years), median weight: 22 kg (12.5-31 kg)] for drug-refractory PAH. For the first time in humans, we performed an unidirectional valved Potts anastomosis in a child with infrasystemic PAH on intravenous epoprostenol who experienced repeated central line infections. Severe postoperative complications occurred in 6 patients (25.0%, all from the surgical group) including 3 early deaths (12.5%) related to low cardiac output. After a median follow-up (FU) of 2.1 years (range, 3 months to 14.3 years, ≥8 years in 7 patients), World Health Organization (WHO) functional class was dramatically improved in the 21 survivors, all being in WHO-functional class 1 or 2 (P < 0.05); none experienced syncope during the FU; none had overt right ventricular failure; mean 6-min walk distance improved from 42.3 ± 10.0% to 81.2 ± 9.7% of adjusted values for age and sex (P < 0.001), BNP/NT-proBNP levels normalized in all; and weaning of intravenous epoprostenol was obtained in all patients who received triple combination as pre-Potts anastomosis therapy. Finally, all survivors caught up to normal growth curves. Arterial oxygen saturation gradient between upper and lower limbs persisted at the last FU (94.7 ± 3.6% vs 81.6 ± 5.1%, P < 0.001). One patient required double lung transplantation 6 years after a surgical Potts shunt. CONCLUSIONS Palliative Potts shunt allows prolonged survival and dramatic, long-lasting improvement in functional capacities in children with severe, drug-refractory PAH. The Potts shunt might be considered as a first surgical or interventional step in the management of children with severe, drug-refractory PAH, leaving the door open for further lung transplantation, if needed.

Surgery for complications of trans-catheter closure of atrial septal defects: a multi-institutional study from the European Congenital Heart Surgeons Association

OBJECTIVE This study aims to analyse the collective experience of participating European Congenital Heart Surgeons Association centres in the surgical management of complications resulting from trans-catheter closure of atrial septal defects (ASDs). METHODS The records of all (n=56) patients, aged 3-70 years (median 18 years), who underwent surgery for complications of trans-catheter ASD closure in 19 participating institutions over a 10-year period (1997-2007) were retrospectively reviewed. Risk factors for surgical complications were sought. Surgical outcomes were compared with those reported for primary surgical ASD closure in the European Association of Cardio-thoracic Surgery Congenital Database. RESULTS A wide range of ASD sizes (5-34mm) and devices of various types and sizes (range 12-60mm) were involved, including 13 devices less than 20mm. Complications leading to surgery included embolisation (n=29), thrombosis/thrombo-embolism/cerebral ischaemia or stroke (n=12), significant residual shunt (n=12), aortic or atrial perforation or erosion (n=9), haemopericardium with tamponade (n=5), aortic or mitral valve injury (n=2) and endocarditis (n=1). Surgery (39 early emergent and 17 late operations) involved device removal, repair of damaged structures and ASD closure. Late operations were needed 12 days to 8 years (median 3 years) after device implantation. There were three hospital deaths (mortality 5.4%). During the same time period, mortality for all 4453 surgical ASD closures reported in the European Association of Cardio-Thoracic Surgery Congenital Database was 0.36% (p=0.001). CONCLUSIONS Trans-catheter device closure of ASDs, even in cases when small devices are used, can lead to significant complications requiring surgical intervention. Once a complication leading to surgery occurs, mortality is significantly greater than that of primary surgical ASD closure. Major complications can occur late after device placement. Therefore, lifelong follow-up of patients in whom ASDs have been closed by devices is mandatory.