Jadwiga Moll

Interventions Complementing Surgery as Part of Multistage Treatment for Hypoplastic Left Heart Syndrome: One Center's Experience

Background Interventional procedures often are used as part of multistage treatment for hypoplastic left heart syndrome (HLHS). This study aimed to evaluate the efficacy of interventions in multistage treatment of HLHS. Methods and Results Between 1999 and 2007, 78 interventions were performed for 58 children with HLHS at different stages of surgical treatment. Group 1, involving 30 interventions for 22 patients with postoperative pulmonary arterial stenosis, showed reduced pulmonary artery pressure and increased vessel diameter. Group 2, involving 15 interventions after the Norwood operation for 12 patients with aortic arch obstruction, showed success for 11 patients. Group 3, involving eight balloon angioplasties of narrowed Glenn anastomosis for seven patients, showed abolition of the pressure gradient between the superior vena cava and the right pulmonary artery. Group 4, involving eight interventional closures of a fenestration for eight patients after a Fontan operation, showed increased oxygen saturation for all eight patients. Group 5, involving seven interventions for six patients in whom venovenous collaterals were closed percutaneously, showed improved oxygen saturation. For Group 6, uncommon miscellaneous interventions were used to stabilize the patients’ condition before the next surgical treatment. Conclusions Interventional procedures play an important role during multistage treatment of HLHS. They allow for a reduction in the number of operations or stabilization of the patients’ condition before the next surgical treatment.

The neoaortic root in children with transposition of the great arteries after an arterial switch operation

OBJECTIVES Neoaortic root changes in children with transposition of the great arteries (TGA) are reportedly risk factors for the development of neoaortic regurgitation (NeoAR). The aims of this study were to assess the neoaortic root diameter and relative proportion in children with TGA after surgical correction and to identify possible correlations with the development of neoaortic insufficiency. METHODS Of the 611 children who had the arterial switch operation performed in the Cardiology Department of the Polish Mothers Memorial Hospital, 172 consecutive patients were qualified for this study. The inclusion criteria were: anatomical correction performed during the neonatal period, more than 10 years of postoperative observation and at least two full echocardiographic examinations. RESULTS NeoAR increased during postoperative follow-up and at the end of the observation period, 76% of the patients had NeoAR (27%-trace, 42%-mild, 7%-moderate and 0.6%-severe). Among the analysed risk factors for NeoAR development, the significant ones were arterial valve discrepancy (OR = 2.05; 95% CI: 1.04-4.02; P = 0.031) and the non-facing commissures (OR = 4.05; 95% CI: 1.34-11.9; P = 0.01). The neoaortic root diameter was not statistically significantly correlated with the presence of NeoAR or with the heart defects associated with transposition. The neoaortic root was initially, on average, 37% (z-score = 1.58) bigger than the aortic root in healthy children. This disproportion increased during the follow-up evaluations to 57% (z-score = 2.09). CONCLUSIONS The neoaortic root in children after the arterial switch procedure develops differently from that in healthy children, but this is not evidently related to NeoAR development or associated heart defects.

Long-Term Outcome of Direct Neopulmonary Artery Reconstruction During the Arterial Switch Procedure

BACKGROUND Neopulmonary stenosis at anastomosis site is one of the most frequent complications after the arterial switch procedure for transposition of the great arteries. The surgical technique is a crucial factor associated with the frequency of stenotic complications. We present the outcomes of direct neopulmonary anastomosis during the arterial switch procedure in patients with simple transposition. This research was to assess the efficacy of this surgical technique based on the incidence of postprocedural supravalvular neopulmonary stenosis (SVPS). METHODS Among 545 patients operated on in our department between 1992 and 2009, the 346 consecutive survivors who had undergone simple transposition in the first month of life were included in this analysis. Switch procedures were performed with direct neopulmonary artery anastomosis in 318 patients (92%); in the remaining 28 (8%), the risk of coronary artery compression required the use of a pericardial patch for pulmonary reconstruction. RESULTS Neopulmonary stenosis occurred in 9 patients (2.6%): 5 had undergone direct neopulmonary reconstruction, and 4 had been treated with a patch. Balloon angioplasty of SVPS was performed twice in 1 patient. No patients required reoperation to treat neopulmonary stenosis. In multivariate analysis (logistic regression), patch reconstruction (odds ratio, 27.5; p=0.001) and nonfacing commissures (odds ratio, 11.1; p=0.004) were correlated significantly with the incidence of SVPS. CONCLUSIONS Direct neopulmonary artery anastomosis during arterial switch is an interesting alternative to patch reconstructions and ensures a good postoperative result with low rates of complications and SVPS.

Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants.

OBJECTIVES Anatomical repair seems an ideal method for the surgical treatment of the anomalous left coronary artery arising from the pulmonary artery (ALCAPA) in infancy. The medium-term outcome has been investigated for infants with ALCAPA following the restoration of a dual-coronary arterial circulation. METHODS Between April 1995 and July 2012, 23 infants with a median age of 4 months underwent surgical repair of ALCAPA in our department. Direct implantation of the anomalous coronary artery into the ascending aorta was feasible in 16 patients. A trap door flap method was used in 5 cases and a tubular extension technique in 2. No infant underwent mitral valve repair at the time of ALCAPA surgery. Left ventricular function and the degree of mitral valve regurgitation were assessed during a 10-year follow-up. RESULTS Four patients died in the early postoperative period, without independent predictors associated with this mortality. During follow-up, improvement in myocardial function occurred in all patients both early and late. There was only one improvement in severe mitral valve regurgitation. Subsequently, 2 children needed mitral valve replacement. There were no early or late reoperations of the reimplanted coronary arteries. CONCLUSIONS Aortic reimplantation is an effective surgical treatment for ALCAPA in infants burdened with a low risk of reoperation due to coronary artery stenosis. There was good potential for myocardial recovery within the first year after surgery. Restoration of the anatomical coronary circulation did not improve mitral valve function in infants with severe preoperative mitral incompetence.

Intra-uterine growth restriction as a risk factor for hypertension in children six to 10 years old

Summary Introduction Intra-uterine growth restriction (IUGR) is present in about 3–10% of live-born newborns and it is as high as 20–30% in developing countries. Since the 1990s, it has been known that abnormalities during foetal growth may result in cardiovascular disease, including hypertension in adulthood. Methods This study evaluated blood pressure parameters (using ambulatory blood pressure monitoring) in children aged six to 10 years old, born as small for gestational age (SGA), and compared them to their healthy peers born as appropriate for gestational age (AGA). Results In the SGA group, an abnormal blood pressure level (prehypertension or hypertension) was present significantly more often than in the AGA group (50 vs 16%, p < 0.01). This relationship also occurred in association with the type of IUGR (asymmetric p < 0.01, symmetric p < 0.05). Conclusion In SGA children, abnormal blood pressure values occurred more frequently than in AGA children.

A 10-year single-centre experience in percutaneous interventions for multi-stage treatment of hypoplastic left heart syndrome

OBJECTIVES The purpose of this paper is to report our 10 years of experience of interventional treatment of patients with hypoplastic left heart syndrome and to focus on the frequency, type, and results of percutaneous interventions during all the stages of palliation, considering the different techniques, devices, and complications. BACKGROUND Constant progress in surgical treatment of congenital heart defects in the last decade has significantly improved the prognosis for children with hypoplastic left heart syndrome. However, morbidity and mortality remain relatively high. Modern interventional procedures complement or occasionally replace surgical treatment. METHODS Between January, 2001 and December, 2010, 161 percutaneous interventions were performed in 88 patients with hypoplastic left heart syndrome. Patients were divided into four groups: (a) before the first surgical treatment including hybrid approach, (b) after first-stage Norwood operation, (c) after second-stage bidirectional Glenn operation, and (d) after third-stage Fontan operation. RESULTS Percutaneous interventions resulted in statistically significant changes in pulmonary artery pressures, vessel diameters, and O2 saturation. Complications occurred in 4.3% of interventions and were related mainly to stent implantation in stenosed pulmonary arteries. CONCLUSIONS Percutaneous interventions may result in haemodynamic stability and reduction in the number of operations. They may result in significant changes in pulmonary artery pressures, vessel diameters, O2 saturation, with a low rate of complications, which are mainly related to stent implantation in the pulmonary arteries.

Prenatal Diagnosis of Partial Anomalous Pulmonary Venous Connection by Detection of Dilatation of Superior Vena cava in Hypoplastic Left Heart

A 27-week fetus was given targeted fetal echocardiography due to an abnormal four-chamber view. A complex heart malformation was seen with two atria, a single ventricle, and aortic and mitral atresia. In addition to intracardiac findings, a dilated superior vena cava (SVC) was seen, suggesting a possible abnormal pulmonary venous connection to the SVC. An increased venous flow (up to 90 cm/s) was recorded as well. The course of pregnancy was uneventful and the baby was delivered vaginally at term. Cardiac surgery was planned but the baby died on 4th day of life. The autopsy findings confirmed both intracardiac anomalies as well as partial anomalous venous connection to SVC, with no apparent obstruction. This is the first report which stresses that dilatation of the fetal SVC may be suspicious for partial abnormal pulmonary venous connection during prenatal life.

Reoperations and catheter interventions in patients with transposition of the great arteries after the arterial switch operation

OBJECTIVES: Reoperations and catheter interventions after the arterial switch operation (ASO) are relatively rare, but their frequency varies among different centres. They significantly impact the postoperative course of children with transposition of the great arteries (TGA). The aim of this study was to assess the frequency of reoperations and catheter interventions in patients with TGA after the ASO and to identify the potential risk factors. METHODS: For this retrospective case review study we included all consecutive 715 patients with TGA who underwent the ASO in the Department of Cardiac Surgery between the years 1991 and 2015. All of the surgical procedures were performed by one cardiac surgery team led by J.J.M., using the same surgical technique with his own specific modifications. RESULTS: The overall early mortality after the ASO was 7.4%; late mortality occurred in 15 cases (2.3%) and the mean clinical follow-up of our cohort was 10.5 years. Early reoperations (<30 days after surgery) were performed in 37 patients (5.1%). Reoperations were performed in 31 patients (4.7% of survivors), and their risk factors were previous early reoperation and left ventricle outflow tract obstruction, while isolated TGA reduced the risk of reoperations. Catheter interventions were performed in 25 patients (3.8% of survivors). In the majority of the cases, the indications for percutaneous procedures were pulmonary stenosis and recoarctation of the aorta. The statistically significant risk factors were aortic arch anomalies associated with TGA and neopulmonary artery anastomosis with a patch, while isolated TGA decreased the risk of reintervention. Freedom from cumulative reinterventions after the ASO was 90.4% at 5 years; 88.0% at 10 years; 86.5% at 15 years and 86.5% from 20 to 25 years. CONCLUSION: The frequency of reoperations and percutaneous interventions in patients with TGA after the ASO remains low. The majority of the procedures are performed because of pulmonary stenosis and recoarctation of aorta. Cardiac anomalies associated with TGA have a significant impact on the incidence of reoperation and reintervention.

Immediate and long-term outcomes of percutaneous transcatheter pulmonary valve implantation

BACKGROUND Transcutaneous pulmonary valve replacement (TPVR) has become an alternative to heart surgery for patients after previous right ventricular outflow tract (RVOT) or pulmonary artery (PA) surgical interventions. The objective was to present immediate and long-term outcomes of trans¬cutaneous pulmonary valve replacement. METHODS Between 06/2009 and 06/2016, 46 patients underwent TPVR. Initial diagnoses included tetralogy of Fallot, common arterial trunk, transposition of great arteries post Rastelli correction, left ventricle outflow obstruction after Ross operation, pulmonary atresia, and isolated dysplastic pulmonary valve stenosis. Thirty eight (78%) patients had previously implanted conduits in the pulmonary position, the rest had either RVOT patch reconstruction (n = 6; 13%) or biological valve implantation (n = 2; 4%). They presented primarily with pulmonary stenosis (n = 18; 39%) or regurgitation (n = 28; 60%). RESULTS All procedures were successful - 44 Melody and 2 Edwards-Sapien valves were implanted. Before each procedure exclusion of potential coronary compression and RVOT prestenting was performed. Significant RVOT systolic gradient reduction (from 35.3 ± 19.5 to 13.5 ± 7.1 mm Hg; p < 0.001) and decrease of right to left ventricle systolic pressure ratio from 0.58 ± 0.18 to mean 0.37 ± 0.1 (p < 0.001) was achieved. Also, in every patient PA-RVOT competence was restored, with minor in¬competence in only a few patients. Post procedure follow-up ranged from 2 to 86 (mean 35.2) months. Follow-up fluoroscopy or chest X-ray revealed 6 stent fractures (2 stent defragmentation - with only 1 significant valve stenosis). CONCLUSIONS Transcutaneous pulmonary valve replacement is a safe procedure with encouraging results, it also enables deferring surgical reintervention in the majority of patients.

Severe Congenital Obstruction of the Left Main Coronary Artery Coexisting With Supravalvular Aortic Stenosis in Williams Syndrome A Dangerous Association

Congenital obstruction of the left main coronary artery is a complicating feature of supravalvular aortic stenosis. We describe an eight-month-old female patient with Williams syndrome, supravalvular aortic stenosis, and branch pulmonary artery stenosis, with concomitant anomaly of severe obstruction of the left coronary artery orifice.