Krzysztof W. Michalak

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.

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.

Fracture and dislocation of a pulmonary stent after cardiac massage.

Following a Glenn procedure, a stent was implanted into the pulmonary artery to relieve stenosis. After the procedure, the patient developed bradycardia and became asystolic. Resuscitation was successful following cardiac massage, but subsequent catheterization revealed the stent to be broken in two, with shift of the distal fragment. The two parts were stabilized and connected by implantation of an additional CP stent.

Successful Late Recruitment of the Occluded Left Main Coronary Artery After Initial Arterial Switch Operation

We describe a surgical technique for late recruitment of the proximally occluded left main coronary artery (LMCA) after initial arterial switch operation, which had been complicated by severe left ventricular dysfunction. This technique allowed for LMCA recanalization, using the patent conal artery branching off the LMCA close to the ostium. It is particularly useful in small children, where surgical options for coronary arteries are limited and associated with a high risk of restenosis. It was successfully used in a two-month-old boy with successful revascularization confirmed by angiography.

Usefulness of Routine Coronary CT Angiography in Patients with Transposition of the Great Arteries After an Arterial Switch Operation

Coronary complications in patients with transposition of the great arteries (TGA) after an arterial switch operation (ASO) are relatively rare, but of all the possible postoperative adverse events, they are potentially the most dangerous. The fate of the coronary arteries, which are transplanted during the neonatal ASO, remains uncertain. There is also no consensus regarding their postoperative evaluation, especially in asymptomatic patients. The aim of this study was to present the early results of routinely performed coronary computed tomography angiography (CCTA) in asymptomatic adolescents and young adults with TGA after an ASO. An initial series of 50 CCTAs performed in asymptomatic patients with TGA after an ASO were evaluated. In each case, a detailed examination of the coronary anatomy, its relationship to the surrounding structures, its exact position in the neoaortic sinus, and the presence of significant coronary abnormalities was performed. The CT scans revealed significant coronary abnormalities in 12 asymptomatic patients: three had acute proximal angulation and stenosis, four had an intra-arterial course, seven had a muscular bridge, one had a left anterior descending artery with an intramuscular course, and one had coronary fistulas to the pulmonary arteries. Additionally, in 25 patients, proximal acute angulation of at least one coronary artery was detected, and four of them had a high ellipticity index. Most of the potentially severe anatomical features were related to the left coronary artery or the left anterior descending artery. CCTA routinely performed on asymptomatic patients with TGA after an ASO provides accurate and useful information for postoperative management. The frequency of coronary anomalies and potentially dangerous anatomical features in this group of patients is high, and their impact on postoperative follow-up remains unknown.

Coronary Artery Anomalies in Patients With Transposition of the Great Arteries and Their Impact on Postoperative Outcomes

BACKGROUND Coronary anomalies are frequently present in children with transposition of the great arteries (TGA). Such anomalies significantly increase the complexity of arterial switch operations and may have an effect on postoperative outcomes. In this study, we aimed to assess the frequency of coronary anomalies in children with TGA and describe their effect on postoperative outcomes. METHODS All patients with TGA who underwent an arterial switch operation between 1991 and 2015 were consecutively enrolled in this study. Patient coronary patterns were obtained from corresponding operative reports and analyzed to determine their associations with adverse postoperative outcomes. RESULTS The study included 715 patients with isolated and complex TGA. Coronary anomalies were present in 33.7% of patients and were significantly correlated with the side-by-side configuration of the great arteries. Coronary sinuses with more than 1 ostium were associated with a significantly increased risk of postoperative death (hazard ratio [HR], 2.58), and coronary arteries with a single ostium were associated with an increased risk of postoperative reinterventions (HR, 2.49). In contrast, the trap-door technique of coronary artery transplantation was associated with a reduced risk of reinterventions during postoperative follow-up (HR, 0.40). Complex coronary anomalies were significantly associated with postoperative coronary events (HR, 2.12). CONCLUSIONS With the exception of patients whose circumflex artery branches off of the right coronary artery, an anomaly that clearly has no effect on postoperative follow-up, patients with unusual coronary patterns are at higher risk for adverse postoperative outcomes than patients with normal coronary anatomy and must be monitored carefully.

12-month assessment of increase in total kidney volume in children and young adults with autosomal dominant polycystic kidney disease – a pilot study

IN T R O D U C T IO N: Autosomal dominant polycystic kidney disease (ADPKD) is the most common type of monogenic kidney disease. It is the cause of ESRD in 5–10% of adult patients who undergo renal replacement therapy. Owing to the increasing use of ultrasonography, occurrence of the disease has been observed among children. M A T E R IA L A N D M ET H O D S: The research group consisted of 19 patients with normal kidney function (12 girls and 7 boys aged 1.8–18.8 at the moment of examination) who suffered from ADPKD. 15 patients met the ultrasonographic criteria of the diagnosis. Although the remaining 4 patients did not meet the criteria, they had a strong family history of ADPKD and underwent USG which revealed at least 2 cysts. For each patient, the Total Kidney Volume (TKV), defined as the sum volume of both kidneys, was juxtaposed with BSA. The children underwent the examination approximately every 12 months. R E S U L T S: The members of the research group manifested a statistically significant annual increase in TKV (in 2015, 296.71 ± 178.67 cm 3 versus 350.38 ± 195.86 cm 3 in 2016, p = 0.019), as well as TKV in relation to body surface (in 2015, 191.23 ± 86.29 cm 3 /m 2 versus 221.15 ± 96.99 cm 3 /m 2 in 2016, p = 0.037). There were no apparent differences in the rate of total TKV increase which would depend either on the patients gender (girls 32.45 ± 51.88 cm 3 /m 2 /year versus boys 25.56 ± 71.00 cm 3 /m 2 /year, p = 0.81), or on the number of renal cysts revealed by USG (< 5 cysts 30.77 ± 61.6 cm 3 /m 2 /year versus ≥ 5 cysts 29.41 ± 58.22 cm 3 /m 2 /year, p = 0.96). C O N C L U S IO N: In children and young adults with ADPKD, the increase in total kidney volume (in relation to BSA) can be observed after a 12-month observation.

Stenting of the right ventricular outflow tract in a symptomatic newborn with tetralogy of Fallot

This case describes a successful percutaneous stent implantation to critical stenosis of the right ventricle outflow tract in a female neonate with tetralogy of Fallot. At the time of the procedure she had poor development of the pulmonary arteries (McGoon and Nakata index 1.45 and 120, respectively). Stent implantation ensured an immediate increase in oxygen saturation level, and the physiological pulsating blood inflow caused good development of the pulmonary arteries during 12 months of follow up (McGoon 2.5; Nacata Index 436). After this time she was qualified for surgery and underwent surgical correction without using a patch or conduit implantation.

Successful percutaneous removal of a fractured stent fragment using a novel stretching method.

We present the case of percutaneous removal of a fractured stent fragment during cardiac catheterisation using a novel stretching method. The procedure was performed in a 9-month-old infant. The small distal fragment of a fractured stent in the aorta was stretched using a two-sided approach - that is, from the carotid and femoral artery. This manoeuvre allowed for the removal of the stretched, linear-shaped stent part through a 6 Fr sheath without any local and general complications.