Images of Extremely Rare Cantrell Phenomenon.

We present a case of a neonate born with prenatal diagnosis of Cantrell syndrome and ectopia cordis. This extremely rare congenital disorder underscores the significant need for multimodality imaging to plan further management. The aim of the study was to present the thoracoabdominal syndrome using a three-dimensional computed tomography angiography. The CT scans confirmed complex intracardiac defects consisting of tetralogy of Fallot, total anomalous pulmonary venous return and persistent left superior vena cava. In conclusion, Cantrell syndrome necessitates a multidisciplinary approach, from the onset of the prenatal diagnosis followed by prompt medical imaging and surgical interventions after birth. The thoracoabdominal wall defect including complete ectopia cordis is an extremely rare disorder with a fatal outcome.

Percutaneous pulmonary valve implantation in a patient with a single pulmonary artery and distal narrowing.

Percutaneous pulmonary valve implantation is established as a safe and effective method of treating patients with disfunction of right ventricular outflow tract. Modifications of this method allow for an increasingly wider use of this less invasive treatment. We present a staged percutaneous pulmonary valve implantation into a single-branch pulmonary artery in a paediatric patient with tetralogy of Fallot after patch repair.

Current practice of care for adolescent and adult patients after Fontan surgery in Poland.

BACKGROUND: The growing number of adult patients after the Fontan operation requires regular surveillance tests in specialized centers. AIMS: Our study aimed to evaluate the current practice of care for Fontan patients in Poland using a multicenter survey. METHODS: Eight centers were included in the study including 5 adult congenital heart disease (ACHD) and 3 pediatric centers for adolescents. To compare the centers and facilitate interpretation of results, the Fontan Surveillance Score (FSS) was developed. The higher score is consistent with better care, with a maximum of 19 points. RESULTS: We included in the study 398 Fontan patients (243 adults and 155 adolescents [aged 14-18 years]). The median FSS was 13 points with variability between centers (interquartile range 7-14 points). Centers providing continuous care from the pediatric period until 18 years of age achieved a higher FSS compared to ACHD centers (median: 14 points vs. 12 points; P <0.001). Most of the patients, both in the ACHD (82.3%) and pediatric centers (89%), were seen annually and had a physical examination, electrocardiogram, and echocardiogram performed at each visit. However, we observed unsatisfactory utilization of tests identifying early stages of Fontan circulation failure (cardiopulmonary exercise tests, cardiac magnetic resonance, liver biochemistry and imaging, detection of protein-losing enteropathy). CONCLUSIONS: Our results showed that there is no unified surveillance approach for Fontan patients in Poland. The practice of care for adults differs from that of adolescents.

Successful complex percutaneous intervention in patient with Fontan circulation and severe heart failure: A case report.

We report the case of a successful complex percutaneous intervention in a patient with Fontan circulation and severe heart failure. The patient presented with cyanosis; Fontan conduit stenosis was detected, and the fenestration was patent. The complex interventional procedure allowed for a long-term stabilization of the patient's condition.

30 years' experience with the arterial switch operation: risk of pulmonary stenosis and its impact on post-operative prognosis.

Pulmonary stenosis is one of the most common complications in patients with transposition of the great arteries after the arterial switch operation. The reason for this is multifactorial and related to the anterior shift of the pulmonary trunk during the LeCompte manoeuvre, the complex suture line required to fill the gaps after harvesting the coronary arteries, and the need for patch implantation to maintain a tensionless anastomosis.We reviewed all patients with transposition of the great arteries operated on at our institute between 1991 and 2020 to establish the frequency of pulmonary stenosis during post-operative follow-up, reinterventions, and reoperations related to pulmonary stenosis and its potential risk factors.During the analysed period, we performed 848 arterial switch operations for simple and complex cases of transposition of the great arteries. The overall early mortality was 6.96%, and the late mortality was 2.53%. Among all study groups, 243 (28.66%) patients developed mild pulmonary stenosis, 43 patients (5.07%) developed moderate, and 45 patients (5.31%) developed severe pulmonary stenosis. During follow-up, 21 patients required interventions related to pulmonary stenosis. Pulmonary reconstruction with patches, aortic arch anomalies, and ventricular septal defects associated with transposition of the great arteries were significant risk factors. Nine patients required reoperation because of pulmonary artery stenosis with patch reconstruction of the pulmonary artery, aortic arch anomalies, and aortic cross-clamping time, increasing the risk of reoperation.Pulmonary stenosis in patients with transposition of the great arteries after the arterial switch operation is a common complication. If significant, it occurs early after surgery and is the most frequent reason for post-operative interventions and reoperations.

Fetal echocardiography and early neonatal balloon valvuloplasty improved overall survival in prenatally detected aortic stenosis over 25 years of tertiary center experience.

PURPOSE: This article aimed to present the factors determining survival and prognosis in fetuses and newborns with critical prenatal aortic stenosis (AS) and to present 26 years of tertiary center experience. METHODS: Study included 87 fetuses with critical AS requiring surgical intervention during neonatal period. All results were expressed as means ± SD, in numbers and percentages. The statistically significant results were those with p < 0.05. RESULTS: An increase in the number of cases of AS was observed in our center along with a decrease in gestational age of our patients during the first echocardiographic exam. The survival rate of newborns was considerably higher when born in due time (p < 0.05) with body weight > 2500 g (p < 0.05). Balloon valvuloplasty performed in the first days after birth occurred to be an optimal solution in these cases. CONCLUSIONS: Fetal echocardiography and special perinatal care with transplacental maternal pharmacotherapy in selected cases and an early neonatal aortic balloon valvuloplasty have shown improvement in survival rate. The most dangerous for the newborn with AS was the first week of postnatal life. It is vital to refer the fetuses with AS to the reference centers which offer the possibility of invasive cardiac intervention on the first day after birth, and it might be an optimal solution.

Lung perfusion scintigraphy in the assessment of pulmonary circulation after completion of surgical treatment of a hypoplastic left heart syndrome (HLHS).

INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is an inborn complex heart malformation. A multi-stage treatment is initiated in a neonatal period with a Norwood surgery. The next step is Glenn surgery - a bidirectional superior cavo-pulmonary anastomosis. At the last stage anastomosis of inferior vena cava (IVC) with the right pulmonary artery (RPA) is formed as a result of a Fontan surgery. The aim of this study was to assess lung perfusion in patients with HLHS after completion of a surgical therapy, using a scintigraphic method. MATERIAL AND METHODS: In 92 patients with HLHS a planar lung scintigraphy in anterior and posterior projections after administration of 99mTc-macroaggregates in activity 18-111MBq was carried out twice (in several day intervals). At first, a radiopharmaceutical was administered to the right extremity in order to assess the lung distribution of blood flowing through the anastomosis of superior vena cava (SVC) with RPA. In the next study, after administration of the tracer to the right lower extremity, the distribution of blood flowing through the anastomosis of IVC with RPA was assessed. The relative percentage of each lung in the total lung perfusion was calculated on a Xeleris workstation using the "Lung perfusion analysis" program. Lung perfusion was considered close to symmetrical when the proportion was in the range of 40-60%. RESULTS: In spite of the fact that mean relative values of distribution of blood flowing through the anastomosis of SVC with RPA to the left lung (LL) and right lung (RL) in the entire study group did not differ significantly: LLmean = 47%; RLmean = 53%, p = 0.14, relative values of perfusion of both lungs were differentiated - in 26% of patients LL was better perfused, in 38% RL was better perfused and in 36% a perfusion of both lungs was similar. The analysis of blood distribution by anastomosis of IVC with RPA showed that the mean relative perfusion of RL was significantly higher than that of LL (70% vs. 30%, p < 0.0000001). No signs of pulmonary emboli were detected. In 13% of studied patients, uptake of the radiopharmaceutical in kidneys was shown indicating the presence of shunt "from right to left". CONCLUSIONS: After completion of surgical treatment of patients with HLHS, differentiated blood supply of the lungs was observed through SVC with RPA anastomosis and a tendency to higher blood supply of RL than LL by anastomosis of IVC with RPA. No signs of pulmonary embolism were detected. The study revealed a "right to left" shunt in some patients.

La fusión de imágenes tridimensionales por TAC y RM antes del cateterismo facilita el implante de stent en defectos cardiacos congénitos / Three-dimensional image fusion of precatheter CT and MRI facilitates stent implantation in congenital heart defects

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Multimodality imaging for no contrast trans-catheter closure of an arteriovenous fistula.

A 6-year-old patient was diagnosed with an arteriovenous fistula in the neck region. Non-invasive three-dimensional imaging, including computed tomography and ultrasonography, was used for guidance of percutaneous closure of the fistula and evaluation of the final outcome.

Glomerular filtration decrease after diagnostic cardiac catheterisation in children with congenital cardiac malformation - the role of serum creatinine, cystatin C, neutrophil gelatinase and urine output monitoring.

INTRODUCTION: Diagnosis of contrast induced-nephropathy (CIN) by a classic renal biomarker such as creatinine concentration can be delayed because of various factors that can influence this marker. Changes in new biomarkers such as neutrophil-gelatinase associated lipocalin (NGAL) and cystatin C are postulated to be more sensitive for recognizing patients prone to CIN-acute kidney injury (AKI). AIM: To investigate the role of NGAL and cystatin C as early biomarkers in the diagnosis of kidney injury after cardiac catheterisation. MATERIAL AND METHODS: The study group consisted of 50 patients with congenital heart malformation admitted for scheduled cardiac catheterisation. The biomarkers serum creatinine, serum NGAL and serum cystatin C were tested at 5 time-points sequentially from start to 48 h after the procedure. RESULTS: Significant changes were noted during the research in the serum creatinine concentration (p < 0.001) and serum NGAL concentration (p < 0.001). CIN-AKI, diagnosed by the modified Schwartz formula, occurred in 16 (32%) patients after 24 h and in 8 (16%) after 48 h. Subsequent analysis showed that serum creatinine significantly rose in the first 2 h of the study with simultaneous reduction in the eGFR. Maximum growth in serum NGAL occurred at 6 h after contrast administration and then returned to the baseline values at 24 h. Serum cystatin C level did not significantly change during the study. CONCLUSIONS: We observed a transient decrease in eGFR and a rise of serum NGAL after 2 h but NGAL was most pronounced at 6 h after the procedure. The potential role of cystatin C as a biomarker of CIN-AKI was not proved.

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.

Stenting of the inter-atrial septum in infants and small children: Indications, techniques and outcomes.

OBJECTIVES: To evaluate the procedural success and outcome of inter-atrial stenting. BACKGROUND: Inter-atrial stenting has been shown to be an effective way to maintain inter-atrial blood flow, however it is considered a high risk procedure, usually performed urgently in patients with significant hemodynamic compromise. METHODS: Between September 2004 and August 2016, inter-atrial stenting was attempted in 29 children. Procedural, clinical, and follow-up data were collected retrospectively. RESULTS: The procedures were completed successfully in 27 patients. Twenty-five procedures were undertaken percutaneously, with the remaining four being performed as hybrid procedures. The patients were considered as high risk for adverse events (82% scored as CRISP 4 and 5) with four deaths during the first 24 hr (14%). Procedural complications occurred in eight patients (28%) with related death in three patients (10%). One further patient died after an uncomplicated technically successful stent implantation performed as a salvage procedure. Procedural complications (71% vs. 14%) and mortality (43% vs. 5%) were higher in those, who weighed 3 kg or less (P < 0.05). Patency of the stents was maintained until planned staged surgery in 22 patients at a mean of 302 days. Three patients underwent further balloon dilation for flow restriction at 58-201 days. In two un-operated patients the stents remained patent at follow-up. One patient with severe pulmonary hypertension died with a patent stent. CONCLUSIONS: Inter-atrial stenting produces reliable patency with a very good success rate. Morbidity and mortality were related to low weight at the time of the procedure.