Unifocalization with pericardial roll technique in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVE: This study aims to compare both the pericardial roll technique with the patch augmentation technique of the unifocalization, and single-stage complete repair with the unifocalization and shunt for the repair of the ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This was a retrospective review of the 48 patients undergoing unifocalization of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries from a single center. Our cohort had two surgical pathways, including single-stage midline unifocalization (n = 40), unifocalization after pulmonary artery rehabilitation by creating an aortopulmonary window or central shunt (n = 8). There were two surgical techniques in single-stage midline unifocalizaton, including widening of the pulmonary arteries with a patch (n = 30), and connecting pulmonary arteries with a pericardial roll (n = 10). RESULTS: A total of 14 (29.2%) of 48 patients underwent single-stage complete repair, 26 patients underwent shunt palliation with unifocalization. Combined early and late mortality was seen in seven patients in those who underwent shunt palliation with unifocalization, while it was seen in one patient in those who underwent a single-stage complete repair (mortality ratio 26.8% vs. 7.1%, p = .22). There was no statistically significant difference between the pericardial roll and patch augmentation techniques in terms of pulmonary artery reintervention (p = .65). Although all pulmonary artery reinterventions were for unilateral pulmonary artery in the roll technique group, 41.7% of reinterventions were for bilateral pulmonary arteries in the pericardial augmentation group. CONCLUSION: Single-stage complete repair of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries has better results than unifocalization with a shunt. In terms of nonvaluable raw material, the use of the pericardial roll technique is a considerable alternative for unifocalization.

Anomalous left coronary artery from the pulmonary artery repair outcomes: Preoperative mitral regurgitation persists in the follow-up.

OBJECTIVE: This study aims to present the midterm outcomes of surgical correction of the anomalous left coronary artery from the pulmonary artery (ALCAPA) with a focus on mitral regurgitation (MR). METHODS: Medical records of 36 ALCAPA patients who underwent surgery in a single center were retrospectively reviewed. RESULTS: There were one (2.7%) neonate, 19 (52.7%) infants, 15 (41.6%) children, and 1 (2.7%) adult patient operated. Coronary reimplantation, tube reconstruction, and coronary artery bypass surgery techniques were performed in 29 (80.5%), 6 (16.6%), and 1 (2.7%) patient, respectively. Sixteen (44.4%) patients had a low ejection fraction and 13 (36.1%) patients had at least moderate MR preoperatively. None of our patients underwent a concomitant mitral surgery during the initial repair. Three and two patients died in the early and late postoperative period, respectively. Two patients underwent reoperation due to MR and pulmonary stenosis, separately. Preoperative MR was the only factor associated with at least moderate MR at the final follow-up (p < .01) and the presence of preoperative moderate or over-moderate MR estimated at least moderate MR at the final follow-up with 100% sensitivity and 80% specificity. CONCLUSION: Although the mitral valve was not repaired in the first operation, the reoperation rate is low. However, a moderate or higher preoperative MR predicted MR at the last follow-up. Performing annuloplasty in such patients can be a strategy to be researched.

High incidence of fever in patients after biointegral pulmonic valved conduit implantation.

OBJECTIVE: To describe our short- and medium-term outcomes using the BioIntegral pulmonic conduit. METHODS: Between August 2018 and September 2019, the BioIntegral pulmonic valved conduit was used for right ventricular outflow tract reconstruction in 48 patients. The data were retrospectively retrieved from the patient charts. RESULTS: The median age at surgery was 36 months (interquartile range [IQR] = 18-62 months). The diagnoses were pulmonary atresia-ventricular septal defect in 28 patients, absent pulmonary valve in four patients, truncus arteriosus in six patients, TGA-VSD-PS in five patients, conduit stenosis in three patients, and left venticular outflow tract obstruction requiring a Ross operation in two patients. In the postoperative short-term follow-up, 15 patients out of 48 had a high fever. Of these, 12 patients had concomitantly elevated C-reactive protein levels. There were no patients with blood culture positivity. The median postoperative length of hospital stay was 14 days (IQR = 8-21 days). The overall mortality was 4% in two patients, one died of right ventricular failure and multiple organ failure and one died of pulmonary embolism. The two patients who died were not among the 15 patients with fever. However, four patients with fever underwent balloon angioplasty for conduit restenosis in their medium-term follow-up. CONCLUSION: There was a high incidence of fever and adverse outcomes in the short-term postoperative follow-up of patients in whom the BioIntegral pulmonic valved conduit was implanted. Caution is advisable in using these conduits until there is convincing evidence about the sterilization and storage standards of these grafts.

Comparison of Doty and McGoon techniques for surgical reconstruction of congenital supravalvular aortic stenosis.

BACKGROUND: We aimed to compare the results of two surgical methods for the treatment of congenital supravalvular aortic stenosis. METHODS: From May 2004 to January 2020, 29 patients underwent surgical repair for supravalvular aportic stenosis in a single centre. The perioperative evaluation of the patients was retrospectively reviewed. RESULTS: Fifteen (51.7%) and 14 (48.2%) patients were treated with the Doty and the McGoon methods, respectively. The median age of our cohort was 4.5 (3.0-9.9) years. Ten (34.5%) patients had Williams-Beuren syndrome, and pulmonary stenosis was observed in 12 (41.3%) patients. The median follow-up time was 2.5 (0.7-7.3) years. On follow-up, five patients had residual stenosis with the McGoon technique and one with the Doty technique (p = 0.05). One patient died early in the post-operative period in the Doty group, and three patients were re-operated on due to restenosis in the McGoon group. Freedom from re-operation in the Doty group at 1, 3, 5, and 10 years was 100%. In the McGoon group, freedom from re-operation rates at the 1-, 3-, and 7-year follow-up were 100, 88.9, and 44.4%, respectively (p = 0.08). CONCLUSION: Our results with both surgical techniques suggest that supravalvular aortic stenosis can be treated with good results. The Doty method provided better relief for the supravalvular aortic segment, considering the residual stenosis and the re-operation rates.

Ligation of patent ductus arteriosus through anterior thoracotomy in preterm infants: a 10-year experience.

OBJECTIVE: The aim of this study is to report on the short-term and mid-term outcomes of preterm infants who underwent patent ductus arteriosus ligation through anterior mini-thoracotomy. METHODS: Data for 103 preterm infants who underwent patent ductus arteriosus clipping through an anterior mini-thoracotomy at the 2nd intercostal space between 2009 and 2019 were retrospectively reviewed. The patients were divided into two groups according to their weight at the time of surgery. The complications, morbidity, and mortality rates of each group were compared at postoperative day 30 and at the end of 1 year after surgery. RESULTS: During the operation, the median weight of the patients was 900 g (IQR 800-1125 g), the median age was 21 days (IQR 14.5-29 days). The lowest body weight was 460 g. In three patients (3%), there was intraoperative bleeding from the patent ductus arteriosus that required transition to median sternotomy. In one patient (1%) a residual patent ductus arteriosus that required reoperation was observed. Twelve patients (12%) died in the first 30 days postoperatively. Six patients (6%) died between the postoperative day 30 and 1 year. There was no statistically significant difference in the rates of mortality, morbidity, and complication between the groups. CONCLUSIONS: Based on our observations of over a hundred preterm infants with patent ductus arteriosus over a decade, ligation through anterior mini-thoracotomy is the main surgical procedure of choice for this patient group in our clinic. Our findings demonstrate the safety of this approach and we believe that it can be successfully replicated in other institutions.

Early outcomes of usage of cryoFORM® probe for cryomaze procedure in congenital heart surgery.

OBJECTIVES: This study aimed to evaluate the early outcomes of patients who underwent a concomitant therapeutic maze procedure for congenital heart surgery. MATERIALS AND METHODS: Between 2019 and 2020, eight patients underwent surgical cryoablation by using the same type of cryoablation probe. RESULTS: Three patients had atrial flutter, two had Wolf-Parkinson-White syndrome, two intra-atrial reentrant tachycardia, and one had atrial fibrillation. Four patients underwent electrophysiological study. Preoperatively, one patient was on 3, two were on 2, five were on 1 antiarrhythmic drug. Six patients underwent right atrial maze and two underwent bilateral atrial maze. Five out of six right atrial maze patients underwent right atrial reduction. Nine different lesion sets were used. Some of the lesions were combined and applied as one lesion. In Ebstein's anomaly patients, the lesion from coronary sinus to displaced tricuspid annulus was delicately performed. The single ventricle patient with heterotaxy had junctional rhythm at the time of discharge and was the only patient who experienced atrial extrasystoles 2 months after discharge. Seven of the eight patients were on sinus rhythm. No patient needed permanent pacemaker placement. CONCLUSION: Cryomaze procedure can be applied in congenital heart diseases with acceptable arrhythmia-free rates by selecting the appropriate materials and suitable lesion sets. The application of cryomaze in heterotaxy patients can be challenging due to differences in the conduction system and complex anatomy. Consensus with the electrophysiology team about the choice of the right-left or biatrial maze procedure is mandatory for operational success.

Repair of a stenotic bicuspid aortic valve by extensive leaflet shaving and commissuroplasty.

The surgical repair of the bicuspid aortic valve in children has been shown to have good outcomes. The repair can be done by the delicate use of native valve tissue without using patch material. The patient is an 8-year-old boy diagnosed with a stenotic bicuspid aortic valve and supravalvular aortic stenosis. He had a balloon valvuloplasty when he was 6 years old. This video tutorial gives detailed information on how to repair the aortic valve by reshaping the aortic cusps and reconstructing the aortic commissures.

Intermediate-term results of Medtronic freestyle valve for right ventricular outflow tract reconstruction in the Ross procedure.

BACKGROUND AND AIM OF THE STUDY: The Ross procedure has become the first choice for aortic valve replacement in children and young adults at many institutions. Since 1997, a lack of availability of homograft valves in Turkey has prompted the use of alternative substitutes for right ventricular outflow tract (RVOT) reconstruction during the Ross procedure. METHODS: Before April 2005, among 20 patients (age range: 14 months to 45 years) at the present authors' institution, the Ross procedure was performed in 14 and a Ross-Konno procedure in six. Sixteen patients underwent RVOT repair using alternative methods for homograft valve replacement. Fourteen patients received a Medtronic Freestyle valve and one patient a Medtronic Contegra bovine jugular vein conduit. An autologous RVOT repair was used in one patient. Ten of the Medtronic Freestyle valve patients were aged <16 years. In all patients who received a Medtronic Freestyle valve echocardiographic evaluations were conducted shortly after surgery and during follow up. RESULTS: There was no early mortality. One patient died from pneumonia after six months, and another (asymptomatic) patient died suddenly at 34 months after surgery. Before hospital discharge the mean peak pressure gradient across the Freestyle valve was 12.1 +/- 11.0 mmHg, and this increased to 24.1 +/- 20.0 mmHg after a mean follow up of 51.2 +/- 6.9 months (range: 6 to 101 months) (p <0.002). Mild pulmonary regurgitation was seen in two patients. One asymptomatic adult patient was reoperated on at another center because of a 60-mmHg echocardiographic peak gradient at four years postoperatively. CONCLUSION: Although long-term follow up is required to explain the durability of the Medtronic Freestyle valve, the present results show that the valve can be used with intermediate-term success in the Ross procedure - and even in children as an alternative - if homograft valves are not available.

Ross-Konno procedure and ostial plasty in a child with homozygous hypercholesterolemia: case report.

Although the Ross procedure is preferred for aortic valve replacement in young and female patients, there are no reported cases of hypercholesterolemic aortic pathology due to homozygous familial hypercholesterolemia. Long-term durability of the pulmonary autograft in patients with postoperative high blood cholesterol levels is of interest. A 14-year-old girl with homozygous familial hypercholesterolemia who underwent the Ross-Konno procedure and left coronary artery ostial plasty was followed for 57 months, with pulmonary autograft function, coronary arteries and lipid profile being monitored. There were no signs of narrowing, insufficiency or calcification of the pulmonary autograft; neither was there any narrowing in the left main coronary ostium. The patient's total cholesterol level was reduced from 897 to 262 mg/dl by use of anti-lipidemic medication and weekly lipid apheresis. Follow up data suggest that a pulmonary autograft may be preferable in children with hypercholesterolemic aortic valvular pathology, as well as in children with aortic valvular diseases of other etiologies.

Two challenging translocation procedures for intramural coronary arteries in the setting of transposition of great arteries.

Translocation of the coronary arteries remains a technical challenge in anatomic correction of transposition of great arteries. Myocardial ischemia related to the difficulties with coronary relocation is an important factor in perioperative and postoperative morbidity and mortality, particularly in the patients with complex coronary artery anatomy. Intramural coronary artery is a rare anatomic variety which may complicate the arterial switch operation in 2% to 4.6% of the reported cases. Even in the hands of experts, the mortality rate may be in twofold in this subset of patients compared with simple transposition of great artery procedures. In this report, 2 successful translocation techniques for intramural coronary arteries in the setting of arterial switch operation are described.