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Valved allografts and xenografts for reconstruction of the right ventricular outflow tract (RVOT) lack durability and do not grow. We report the first clinical use of a completely bioabsorbable valved conduit (Xeltis pulmonary valve - XPV) in children. Twelve children (six male), median age five (two to twelve) years and median weight 17 (10 to 43) kg, underwent RVOT reconstruction with the XPV. Diagnoses were: pulmonary atresia with ventricular septal defect (VSD) (n = 4), tetralogy of Fallot (n = 4), common arterial trunk (n = 3), and transposition of the great arteries with VSD and pulmonary stenosis (n = 1). All had had previous surgery, including prior RVOT conduit implantation in six. Two diameters of conduit 16mm (n = 5) and 18mm (n = 7) were used. At 24 months none of the patients has required surgical re-intervention, 9 of the 12 are in NYHA functional class I and three patients in NYHA class II. None of the conduits has shown evidence of progressive stenosis, dilation or aneurysm formation. Residual peak gradient of >40 mm Hg was observed in three patients, caused by kinking of the conduit at implantation in 1 and distal stenosis in the peripheral pulmonary arteries in 2 patients. Five patients developed severe pulmonary valve insufficiency (PI); the most common mechanism was prolapse of at least one of the valve leaflets. The XPV conduit is a promising innovation for RVOT reconstruction. Progressive PI requires however an improved design (geometry, thickness) of the valve leaflets., (Copyright © 2021. Published by Elsevier Inc.)

The Mustard operation for transposition of the great arteries is associated with good long-term survival. Typical complications at follow-up include progressive loss of sinus node function requiring permanent pacemaker implantation, and systemic venous pathway obstruction often precluding a transvenous approach to pacing. We report on 7 patients (median age 14.1; range 5-19) with bradyarrhythmia requiring permanent pacemaker implantation with associated stenosis (n = 6) or occlusion (n = 1) of the superior vena cava, in whom stent implantation relieved the obstruction and facilitated subsequent transvenous permanent pacing. In five of them stenting and pacemaker implantation were performed during a single procedure; two patients underwent elective pacemaker implantation 6 weeks later. In one patient the pacemaker had to be explanted due to pacemaker pocket infection. In the others the follow-up has been uneventful, with excellent chronic pacing thresholds and appropriate sensing. Two patients have had their generator replaced electively.We conclude that stenting of the SVC stenosis allows implantation of transvenous pacemaker leads with good intermediate term results in patients with a Mustard operation for transposition of the great arteries.

Objective: The arterial switch operation was introduced in 1976 to correct transposition of the great arteries and has since replaced atrial palliation. This study examines the long-term outcomes of the arterial switch operation., Methods: Included in the study were 195 patients who underwent the arterial switch operation from 1977 through June 2000. Patients were evaluated for functional class, pulmonary stenosis, left ventricular function, arrhythmias, aortic sequelae, and coronary disease., Results: Overall perioperative mortality was 15%, but rates dropped in the last 5 years to 4% for complex transposition and 0% for simple transposition. Of 151 survivors, 2 died late; 1 died of persisting pulmonary hypertension and 1 died of ventricular fibrillation after coronary pathologic changes. At last follow-up, a total of 145 patients were in New York Heart Association functional class I and 4 patients were in class II. The most frequent complication was pulmonary stenosis, necessitating 45 reinterventions in 26 patients. Left ventricular dysfunction was noted in 5 patients. Arrhythmias were seen in 5 patients: 2 patients had ventricular fibrillation (1 died and 1 required a defibrillator implantation), 1 had sick sinus syndrome, 1 had atrial flutter, and 1 had a single attack of supraventricular tachycardia. Aortic valve incompetence was absent or trivial in 146 patients, 3 had mild incompetence, 1 had moderate incompetence, and 1 had severe incompetence. Coronary sequelae were found in 5 of the 61 patients who underwent angiography., Conclusion: Long-term clinical outcome of the arterial switch operation is good, and perioperative mortality is now low. Morbidity is dominated by pulmonary stenosis and coronary artery disease, with the potential for lethal arrhythmias.

Objective: To examine the results of the arterial switch operation which is used to correct the transposition of the great arteries., Design: Descriptive., Method: In the period May 1977-October 2000 195 patients at the Wilhelmina Children's Hospital in Utrecht, the Netherlands, underwent an arterial switch operation. The patient group consisted of 132 boys and 63 girls, with a median age at surgery of 8 days (range: 0-1976). In accordance with the protocol, the postoperative follow-up consisted of echocardiography and an ECG. Further investigations were performed on indication. Additional data were collected from the status reports. The mean follow-up was 8.0 years (range: 0.04-22.17)., Results: The overall peri-operative mortality was 15% (30/195). Initially this figure was about 50%, in the 1980s it was 15% and from 1996 onwards it was 4%. Of the 151 surviving and traceable patients, 2 patients died years later (1.3%). Of the remaining 149 patients, 145 were classified in New York Heart Association class I and 4 in class II. Due to pulmonary stenosis, 45 reinterventions were required in 26 patients (/149; 17%). Left ventricle dysfunction occurred in 5 patients (3%), arrhythmias in 5 patients and aorta abnormalities in 5 patients. Coronary artery abnormalities were found in 5 of the 61 patients who underwent angiography (8%)., Conclusion: Peri-operative mortality decreased to 4% and late mortality was 1.3%. The long-term clinical outcome of the arterial switch operation was good. Morbidity was predominantly caused by pulmonary stenosis and late coronary obstruction.

Objective: Concerns have been voiced about possible dilation and insufficiency of the neo-aortic valve after the arterial switch operation (ASO)., Aims: To determine growth of the neo-aortic valve and the aortic anastomosis after ASO and the prevalence of insufficiency or stenosis., Patients and Methods: Since 1977, 144 consecutive patients (pts) underwent ASO for transposition of the great arteries (TGA). Median follow-up was 8.65 years (0.1--22.5 years). Simple TGA was present in 97 pts and 47 had TGA with ventricular septal defect (VSD). Detailed echocardiography included measurements of aortic diameter at four levels. The 608 measurements were compared with published normal values., Results: The mean aortic valve z-score was 1.5, without significant change with age (P=0.75). Under 4 months, mean valve z-score was 0.63+/-2.20, between 5 and 12 months 2.56+/-2.30 (P<0.0001). Gradual growth occurs thereafter. The aortic sinus follows an identical growth pattern. The aorta at the anastomosis, is initially smaller than normal (z-score -0.64). After 4 months the z-score is 0.83, followed by continued growth of 0.1 z-score per year. At the last visit, the aortic valve z-score was above 2 in 51 patients, between -2 and 2 in 72 and less than -2 in six patients, none of whom had a flow velocity above 2 m/s. z-score of patients with VSD remained above those without VSD (P<0.0001). Aortic insufficiency was grade 2/4 in three patients, grade 3/4 in one and grade 4/4 in one. No patient developed aortic stenosis., Conclusion: After ASO the neo-aortic valve and sinus are larger than normal, representing the natural size difference in the prenatal situation and influence of associated cardiac malformations. In the first year of life, rapid dilatation of the new aorta is observed, followed by growth towards normalization of the valve and sinus size. Stenosis at the anastomosis was not observed. Aortic dilatation by itself is rarely associated with significant insufficiency.

A 2-month-old infant presented with acute onset of heart failure, having previously undergone anatomical repair of transposition of the great arteries and ventricular septal defect (VSD). Echocardiography demonstrated aneurysmal dilation of the native pericardial patch used for VSD closure, resulting in right ventricular inflow obstruction. The pericardial patch was excised, and the VSD closed using a GoreTex patch.

Background: Abnormal coronary artery anatomy is reported to have a significant influence on the outcome of the arterial switch operation. This study examines the impact of coronary anatomy and the occurrence of late coronary obstruction on left ventricular (LV) function and long-term outcome., Methods: Coronary artery anatomy, of 170 patients after arterial switch operation (1977-1999), was determined based on operative reports and pre-operative aortograms. Current status was evaluated using ECGs, echocardiograms, scintigraphy, and post-operative coronary angiograms., Results: In 133/170 patients, coronary artery anatomy consisted of an anterior descending (LAD) and circumflex artery (Cx) from the left sinus and the right coronary artery (RCA) from the right or posterior sinus. The left coronary had an intramural initial course in two of these patients. Fifteen patients had the LAD from the left and Cx and RCA from the right sinus; eight had LAD and RCA from one sinus and Cx from the other; four had single ostium; and three had three separate ostia. Four patients had complex patterns and four patients had a supra commissural coronary. To date, follow-up angiography was performed in 59 patients. Surgical coronary sequellae were found in five patients. Two patients had an occluded left ostium. Initially, they were asymptomatic but showed polymorphic ventricular extrasystoles on ECG and moderate LV dysfunction with large irreversible perfusion defects on scintigraphy. Both patients developed ventricular fibrillation at the age of 14 years. One patient did not survive. The other patient required implantation of a defibrillator. One patient has an occluded RCA, one patient has stenosis of the right ostium and one patient has multiple tortuous collaterals without obstruction of a major branch. In the latter three patients, coronary sequellae were not suspected on ECG, echo, or scintigraphy and were only found on follow-up angiography. Retrograde collateral flow was noted in all three occluded coronaries. LV dysfunction, with normal coronaries, was noted in three patients. All, of these patients, had peri-operative ischaemia suggesting failure of myocardial protection. Two are now asymptomatic with mild LV dysfunction. One patient continues to have severe myocardial dysfunction and secondary aortic insufficiency. A Ross-like procedure was performed placing the original aortic valve in the neo-aortic root. Coronary artery anatomy did not influence early survival or late coronary sequellae., Conclusion: Abnormal coronary anatomy was not a determinant of outcome in our study. Surgical coronary obstruction is independent of original anatomy. It can be almost silent and is potentially fatal. Follow-up angiography must be considered in all patients after the arterial switch operation.