Dominique Piot

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.

Surgery for congenital mitral valve disease in the first year of life

Between 1980 and 1993, 20 patients less than 1 year of age underwent operations for congenital mitral valve disease. Ten patients had congenital mitral incompetence and 10 had congenital mitral stenosis. Mean age was 6.6 +/- 3.4 months and mean weight was 5.6 +/- 1.5 kg. Atrioventricular canal defects, univentricular heart, class III/IV hypoplastic left heart syndrome, discordant atrioventricular and ventriculoarterial connections, and acquired mitral valve disease were excluded. Indications for operation were intractable heart failure or severe pulmonary hypertension, or both. Associated lesions, present in 90% of the patients, had been corrected by a previous operation in seven. In congenital mitral incompetence there was normal leaflet motion (n = 3), leaflet prolapse (n = 2), and restricted leaflet motion (n = 5). In congenital mitral stenosis anatomic abnormalities were parachute mitral valve (n = 4), typical mitral stenosis (n = 3), hammock mitral valve (n = 2), and supramitral ring (n = 1). Mitral valve repair was initially performed in 19 patients and valve replacement in one with hammock valve. Concurrent repair of associated lesions was performed in 12 patients. The operative mortality rate was zero. There were six early reoperations in five patients for mitral valve replacement (n = 4), a second repair (n = 1), and prosthetic valve thrombectomy (n = 1). One late death occurred 9 months after valve replacement. Late reoperations for mitral valve replacement (n = 2), aortic valve replacement (n = 1), mitral valve repair (n = 2), subaortic stenosis resection (n = 1), and second mitral valve replacement (n = 1) were performed in five patients. Actuarial freedom from reoperation is 58.0% +/- 11.3% (70% confidence limits 46.9% to 68.9%) at 7 years. After a mean follow-up of 67.6 +/- 42.8 months, 94% of living patients are in New York Heart Association class I. Doppler echocardiographic studies among the 13 patients with a native mitral valve show mitral incompetence of greater than moderate degree in one patient and no significant residual mitral stenosis. Overall, six patients have mitral prosthetic valves with a mean transprosthetic gradient of 6.2 +/- 3.7 mm Hg. These results show that surgical treatment for congenital mitral valve disease in the first year of life can be performed with low mortality. Valve repair is a realistic goal in about 70% of patients and possibly more with increased experience. Reoperation rate is still high and is related to complexity of mitral lesions and associated anomalies, but late functional results are encouraging.

Surgical approaches for double-outlet right ventricle or transposition of the great arteries associated with straddling atrioventricular valves☆☆☆★★★♢♢♢♦

The surgical management of patients with double-outlet right ventricle or transposition of the great arteries and straddling atrioventricular valves remains a subject of controversy. Biventricular repair has theoretic advantages because it establishes normal anatomy and physiology. In some instances, however, it seems to carry too high operative risk, and a univentricular heart repair is preferred. Since 1984, we have operated on 34 patients with double-outlet right ventricle (n = 15) or transposition of the great arteries (n = 19) with isolated straddling tricuspid valve (n = 17), isolated straddling mitral valve (n = 9), both mitral and tricuspid straddling (n = 2), or abnormal insertion of tricuspid (n = 7) or mitral (n = 2) chordae in the left ventricular outlet, precluding an adequate tunnel construction. Straddling was categorized according to the location of the papillary muscle insertion in the opposite ventricular chamber: type A, on the edge of the ventricular septal defect (n = 14); type B, on the opposite side of the ventricular septum away from the edge of the defect (n = 8); type C, on the free wall of the opposite ventricular chamber (n = 8). Abnormal chordal insertions were classified according to the location of their attachments around the edges of the defect. Three types of chordal distribution were identified: on the aortic conus, on the pulmonary conus crossing the ventricular septal defect, or around the defect closing it like a curtain. All but three patients had two ventricles of adequate size. Sixteen patients underwent palliation. Median age at the definitive operation was 6.5 months (range 1 to 130 months). Thirty patients underwent a biventricular repair and four had a univentricular repair. Biventricular repair was achieved by an arterial switch operation in 18 patients and by tunnel construction from the left ventricle to the aorta in 12. In isolated straddling of types A and B, the ventricular septal defect was closed by adjusting the septal patch on the ventricular side above the straddled papillary muscle. In type C, the patch was sewn over the papillary muscle by applying it on the septum. In double straddling, the ventricular septum was incised between the two papillary muscles, and an ellipsoid patch was used to reconstruct the septal defect, directing each subvalvular apparatus into its own ventricular chamber. When the abnormal chordae in the left outflow tract inserted on the aortic or pulmonary conus, the conus was incised and tailored to make a flap, leaving an unobstructed left ventricular outflow tract. In two patients the subvalvular apparatus was resected and reattached to the patch. Curtainlike chordae were a contraindication to biventricular repair in double-outlet right ventricle but not in transposition. There were four early deaths and one late death, all occurring in the group having biventricular repair. Death was due to myocardial ischemia (n = 1), right ventricular hypoplasia (n = 1), pulmonary hypertension (n = 1), and residual subaortic stenosis (n = 1). Two patients had moderate to severe postoperative atrioventricular valve incompetence, caused by a cleft in the mitral valve in one patient. Three patients were reoperated on for subaortic stenosis (n = 1), pulmonary stenosis (n = 1), and mitral regurgitation (n = 1). Mean follow-up of 30.7 +/- 19.4 months was achieved in the survivors. All but one patient (univentricular repair) were in New York Heart Association class I, without atrioventricular valve incompetence. Actuarial survival at 4 years was 85.3% +/- 3%. We conclude that straddling or abnormal distribution of chordae tendineae of the atrioventricular valves does not preclude biventricular repair in double-outlet right ventricle or transposition of the great arteries provided that the ventricles are of adequate size. Curtainlike abnormal tricuspid chordae remain a contraindication to biventricular repair in double-outlet right ventricle.

Early liver transplantation is crucial in children with liver disease and pulmonary artery hypertension

BACKGROUND/AIMS Early liver transplantation is crucial in children with liver disease and pulmonary artery hypertension. Some severe pulmonary vascular anomalies associated with portal hypertension disappear after isolated liver transplantation. Evolution of pulmonary artery hypertension due to plexogenic arteriopathy is controversial, as this association is still considered a contraindication to isolated liver transplantation. Outcome of pulmonary hypertension after isolated liver transplantation is reported in three patients with portal hypertension. METHODS After echocardiographic diagnosis, the patients had a complete hemodynamic exploration, and two had a lung biopsy. After liver transplantation, the survivors had echocardiographic follow up and a second hemodynamic exploration. RESULTS In two children, pulmonary pressures and resistances returned to near-normal values 1 and 6 years after successful isolated liver transplantation. The third patient, with the most severe arteriopathy, had to wait 1 year for a donor, and the attempted transplantation was complicated by ventricular tachycardia; death occurred 2 days after surgery. CONCLUSIONS Liver transplantation can reverse pulmonary artery hypertension due to high pulmonary resistances complicating liver disease with portal hypertension, provided it is carried out at an early stage. Early detection of pulmonary hypertension by systematic echocardiography may thus be crucial in these children with portal hypertension.

Surgical management of atrio ventricular septal defects with normal caryotype.

OBJECTIVE Atrio ventricular septal defects (AVSD) with normal caryotype represent in average 25% of AVSD. They constitute a more complex group of patients characterized by frequent left sided heart obstructive lesions, raising the problem of the appropriate indications between biventricular and univentricular procedures. METHODS Sixty-nine consecutive patients, who had AVSD with normal caryotype underwent surgery. According to the anatomical complexity there were 22 intermediate AVSD, 36 complete AVSD and 11 complex AVSD. Associated lesions were present in 68% of the patients including left sided heart anomalies in 57%. According to the size of the left ventricle (LV) evaluated on the LV/RV end diastolic diameter ratio calculated at 2D echocardiogram: right ventricular (RV) dominance was found in 29%; with border line LV in 13 patients and truly hypoplastic LV in 7 patients. Biventricular repair was always favored in case of border line LV and precluded when the LV/RV ratio was less than 0.33. RESULTS There were 57 biventricular repairs with 10 years actuarial survival of 70 +/- 8% and respectively, 100% in the complex AVSD, 86% in the intermediate AVSD and 51% in the complete AVSD (P = 0.006). The risk factors for biventricular repair were the association to a subaortic stenosis (P = 0.01) and the severity of the mitral valve lesions (P = 0.03) that led to 38% reoperation. There were 12 univentricular palliation with 10 years survival of 66 +/- 14%. The risk factor for univentricular palliation was the association to a severe pre-operative mitral regurgitation (P = 0.005). CONCLUSIONS Biventricular repair should be precluded in patients presenting with subaortic stenosis. Severe mitral valve anomalies lead to elevated mortality and morbidity with frequent reoperations. Univentricular repair might have larger indications and cardiac transplantation might be considered in patients with truly hypoplastic LV presenting with severe pre-operative AV valve regurgitation.

Transcatheter Closure of Large Atrial Septal Defects Feasibility and Safety in a Large Adult and Pediatric Population

Background—Data are needed on the safety and efficacy of device closure of large atrial septal defects. Methods and Results—Between 1998 and 2013, 336 patients (161 children <15 years) with large, isolated, secundum atrial septal defects (balloon-stretched diameter ≥34 mm in adults or echocardiographic diameter >15 mm/m2 in children) were managed using the Amplatzer device, at the Marie Lannelongue Hospital. Transthoracic echocardiographic guidance was used starting in 2005 (n=219; 65.2%). Balloon-stretched diameter was >40 mm in 36 adults; mean values were 37.6±3.3 mm in other adults and 26.3±6.3 mm/m2 in children. Amplatzer closure was successful in 311 (92.6%; 95% confidence interval, 89%–95%) patients. Superior and posterior rim deficiencies were more common in failed than in successful procedures (superior, 24.0% versus 4.8%; P=0.002; and posterior, 32.0% versus 4.2%; P<0.001). Device migration occurred in 4 adults (2 cases each of surgical and transcatheter retrieval); in the 21 remaining failures, the device was unreleased and withdrawn. After a median follow-up of 10.0 years (2.5–17 years), all patients were alive with no history of late complications. Conclusions—Closure of large atrial septal defects using the Amplatzer device is safe and effective in both adults and children. Superior and posterior rim deficiencies are associated with procedural failure. Closure can be performed under transthoracic echocardiographic guidance in experienced centers. Early device migration is rare and can be safely managed by device extraction. Long-term follow-up showed no deaths or major late complications in our population of 311 patients.

Transposition of the great arteries complicated by tricuspid valve incompetence

BACKGROUND Tricuspid valve insufficiency secondary to structural anomalies of the valve itself or to an iatrogenic complication of the Rashkind procedure is very rarely associated with transposition of the the great arteries. This condition represents an interesting perioperative challenge. Rapid restoration of the tricuspid valve to a low-pressure system by arterial switch operation associated with tricuspid repair should theoretically improve the outcome in terms of myocardial and valve function. METHODS Thirteen of 839 patients who underwent an arterial switch operation for various forms of transposition of the great arteries presented with moderate to severe tricuspid insufficiency. Three of them had a ventricular septum defect. Nine experienced severe cardiac failure with profound hypoxemia. Ventilatory support was necessary in 7, 6 had renal or hepatic dysfunction, and 5 had coagulation disorders. Inotropic support was started preoperatively in 8 patients. RESULTS Tricuspid lesions were as follows: primary annular dilatation and lack of coaptation at the commissural level (n = 1), straddling tricuspid valve (n = 1) redundant tricuspid valve tissue leading to left ventricular outflow tract obstruction (n = 1), small cleft of the septal leaflet (n = 1), and dysplastic valve tissue with juxtacommissural regurgitation (n = 1). In 8 patients, the cause of the tricuspid valve insufficiency was most probably an iatrogenic lesion, with rupture of the papillary muscle (n = 2), rupture of the chordae (n = 1), or tear of the anterior leaflet (n = 5), whereas no clear cause could be found in 1 patient. Repair consisted of the arterial switch operation associated with tricuspid valve repair in 10 patients. In 2 patients with only discrete anomaly and in 1 without a clear cause of tricuspid regurgitation, no valve repair was performed. Three patients had their ventricular septal defect closed. There were only one early and one late death, both not related to the tricuspid lesions. Late postoperative (mean, 6.5 years) evaluation revealed normal left ventricular function in 10, with no tricuspid incompetence in 7 and trivial tricuspid insufficiency in 3. CONCLUSIONS Restoration of an incompetent tricuspid valve in a low-pressure system by the arterial switch operation combined with valve repair provides good ventricular and valvar results. Preoperative management and appropriate timing of operation seem to be of utmost importance.