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Pulmonary vein (PV) stenosis is a rare disease with high recurrence and mortality rates. Objective. This study was aimed to evaluate the diagnostic accuracy of echocardiography versus cross-sectional modalities. Material and methods. Our study includes 11 consecutive patients who underwent a comprehensive PV anatomy assessment between April 2018 and June 2019 at Ukrainian Children’s Cardiac Center, Kyiv. Echocardiographic studies of each pulmonary vein followed by computed tomography (CT) or magnetic resonance imaging (MRI) were performed in all patients. A total of 27 CT/MRI studies were performed. Results. There were four patients whose echocardiography findings were completely confirmed by CT/MRI method. In some patients significant stenosis was underestimated by echocardiography. Sensitivity and specificity were 86.4 and 88.9 for echo, as well as 100 and 94.4 for CT/MRI, respectively. Conclusion. This study recognizes the limitations of echocardiography as an imaging tool for pulmonary veins morphology assessment. Cross-sectional studies overcome these limitations and provide excellent morphological as well as functional (MRI) evaluation of pulmonary veins.

Objectives We analysed a large series of truncus arteriosus repairs with a focus on early and late outcomes. Methods Ninety-seven consecutive patients who underwent truncus arteriosus repair (1997-2017) were included retrospectively. Univariable analysis for mortality and reintervention was performed. Results The early mortality rate decreased from 45% (1997-2007; 14/31) to 4.5% (2008-2017; 3/66) (P = 0.001). Repair beyond the neonatal period (P = 0.03) and direct connection for right ventricular outflow tract reconstruction (P = 0.001) were associated with early death by univariable analysis. Overall survival was 68 ± 6.0% at 15 years; a majority of the deaths (90%; 9/10) occurred within the first year after repair. Freedom from the first and second conduit reoperations at 10 years was 22.9% and 89%, respectively. Freedom from truncal valve (TrV) reoperation was 83.9% at 15 years. Initial TrV insufficiency ≥ moderate was associated with a TrV reoperation (P = 0.008) with freedom from TrV reoperation in this subgroup of 58.3% at 10 years. Freedom from TrV reoperation for quadricuspid and tricuspid TrVs was 66.8% and 93.8% at 10 years with 100% for bicuspid TrVs at 8 years. At the last follow-up, 98.5% (69/70) were in New York Heart Association functional class I-II. Conclusions In the current era, truncus arteriosus can be repaired with a low early mortality rate and a good long-term outcome. A significant reintervention burden still persists. Direct connection is associated with early mortality.

Pulmonary vein stenosis (PVS) is a rare but serious condition characterized by obstruction of extrapulmonary segments of pulmonary veins which leads to progressive pulmonary hypertension. Objective. In this study we aimed to determine relationship between clinical features and histopathologic findings in patients with PVS. Material and methods. We retrospectively reviewed 34 consecutive patients who underwent PV stenosis repair. Surgical wedge biopsy specimens were collected intraoperatively in 11 patients and reviewed using light microscopy. Results. Affected pulmonary veins in patients with primary PVS were characterized by diffuse stenosis extending into the lung parenchyma. In post-surgical group stenosis was found in a limited segment of pulmonary vein at its ostium. Microscopically, abnormal intimal proliferation was identified in both patient groups. Scaring was predominant finding in patients with post-surgical PVS. Conclusion. In patients with PVS, pathophysiological mechanism influences the severity and extent of clinical manifestations. A comprehensive understanding of this mechanism may improve results of the treatment.

Pseudocoarctation of the aorta is an uncommon congenital entity characterized by juxtaductal elongation and tortuosity of the aortic arch. It is known to be associated with aneurysm formation. Our patient is a 7.7-month-female infant followed prenatally by magnetic resonance imaging that detected this anomaly. Subsequently, postnatal preoperative computed tomography angiography was confirmatory. The operation comprised resection of the aneurysmal portion of the aorta and extended end-to-end reconstruction, without prosthesis usage. The left subclavian artery was reimplanted into the left carotid artery. The postoperative course was uneventful. Computed tomography aortography and transthoracic echocardiography demonstrated no residual stenosis at six months of follow-up.

A neonate with antenatally diagnosed severe aortic valve stenosis, left ventricular outflow tract obstruction, left ventricular endocardial fibroelastosis, and severe mixed mitral valve disease was admitted to our center for balloon aortic valvuloplasty. On day 49, we performed a Ross-Konno procedure and mitral valve replacement on the baby, whose weight was 3.4 kg. Right ventricle-to-pulmonary artery continuity was restored with a handmade trileaflet Gore-Tex conduit. We used a handmade cylindrical prosthesis made from decellularized equine pericardium in the mitral position. We observed improvement in left ventricular function and good performance of aortic, pulmonary, and mitral prostheses at four months of follow-up.

Objectives This article reports the safety and efficacy of a morphology-based algorithm for the surgical management of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries. Methods A total of 83 patients were operated from 2007 to 2014 using 3 surgical approaches: one-stage total repair, one-stage unifocalization with central shunt and delayed total repair and multistage unifocalization with subsequent total repair. Patients were divided into 2 groups depending on the surgical strategy used to choose the surgical approach. In Group I (2007-09), the surgeon-preferred procedure (71%-one-stage total repair) was used. In Group II (2010-14), a surgical algorithm based on pulmonary arterial tree morphology was applied. Results Median follow-up was 5.04 years and 98% complete. The overall survival rate was 92.59%. Cumulative operative mortality decreased from 10.7% in Group I to 0% in Group II ( P = 0.036). Successful complete repair was performed in 23 (82%) of 28 patients in Group I (median follow-up, 7.99 years) and in 33 (60%) of 55 patients in Group II (median follow-up, 3.85 years). The difference in total survival was not significant between Groups I and II but was markedly influenced by the presence of the 22q11 deletion. The survival of 23 patients with confirmed 22q11 deletion was 73.91%; the survival of 28 patients with confirmed absence of the 22q11 deletion was 100%; and the survival of 32 patients not tested for the 22q11 deletion was 100% ( P = 0.0001). Conclusions Total survival was significantly lower in patients with the 22q11 deletion. Surgical management based on preoperative pulmonary arterial anatomical features improves early surgical results.

We assessed morbidity after 2 management strategies for arterial switch operation (ASO) in a single institution: first hours of life surgery and conventional approach. From September 2009 to September 2014, 346 consecutive patients who underwent ASO were enrolled. The study group included 92 patients who underwent ASO in the first 24 hours after birth (group I). The control group consisted of 254 patients who underwent ASO after 24 hours of life in the conventional way (group II). Three outcomes were analyzed: postoperative ventilation time (VT), post-extubation hospital length of stay (peLOS), and a composite morbidity index, defined as postoperative VT + peLOS + occurrence of selected major complications. Overall 30-day survival was 98% (2 [2%] group I vs 5 [2%] group II, P = 1.000). Fifty (13.3%) major complications were observed: 14 (15%) in group I and 36 (15%) in group II (P = 0.635). Although peLOS and morbidity index did not differ significantly between groups, postoperative VT (42 hours [24, 67] vs 27 hours [22, 47], P = 0.008) and total hospital stay were significantly longer in group II (18 days [10, 19] vs 14 days [12, 18]). A median volume of 80 mL (60-100 mL) of autologous umbilical cord blood was collected (80 mL, group 1 vs 60 mL, group II; P = 0.090). Homologous blood cell transfusion was avoided in 70 patients (78%) in group I and in 13 patients (6%) in group II (P 0.001). Arterial switch operation during the initial 24 hours of life has similar outcomes in view of morbidity and mortality to the conventional approach.

Background: This study evaluates the hemoglobin-oxygen relationship and tissue perfusion during cardiopulmonary bypass (CPB) in neonates undergoing open-heart surgery using autologous umbilical cord blood or donor blood components. Methods: We compared perioperative hematocrit (Hct), fetal hemoglobin (HbF), p50O2, pH, pCO2, serum lactate, duration of mechanical ventilation and intensive care unit (ICU) length of stay in neonates undergoing open-heart surgery using autologous umbilical cord blood (Group I, N=45) or donor blood components (Group II, N=65). The groups were similar with respect to diagnosis, weight, type of procedure, duration of CPB and duration of myocardial ischemia. Results: Mean p50O2 was significantly lower in Group I during CPB (19.7 vs. 22.3 mmHg, p=0.004) and at the end of CPB (20.1 vs. 22.8 mmHg, p=0.003). Median peak lactate during CPB was higher in Group I (4.8 vs. 2.2 mmol/l, p50O2 during CPB in Group I (β=0.88, p=0.002), but not Group II. Bodyweight, Hct, duration of CPB, pre-CPB lactate level and pCO2 affected peak lactate level during CPB. Although mean duration of ventilation was longer in Group II (mean 51 vs. 43, p=0.004), the groups experienced similar duration of ICU stay (5.8 vs. 5.9 days, p=0.280). Conclusions: Despite the fact that the oxyhemoglobin dissociation curve is shifted leftward in patients who receive autologous umbilical cord blood, tissue oxygen delivery appears to be preserved in neonates who undergo open-heart surgery using autologous umbilical cord blood.

A 5-hour-old boy underwent arterial switch operation and totally anomalous pulmonary venous connection repair. Subsequently developed severe pulmonary venous stenosis was successfully treated with hybrid balloon angioplasty.

A 26-year-old woman underwent tetralogy of Fallot repair and classic Blalock-Taussig shunt (BTS) ligation. On postoperative day 4 she developed severe left-sided hemiplegia due to cerebral infarction. Thrombi in the BTS stump, right brachiocephalic artery, and its branches were removed surgically and the BTS stump was resected. The patient gradually recovered from hemiplegia and is leading a normal life with moderate left hemiparesis and is in good functional status 4 years after the event. An aggressive approach in treating this complication was crucial to prevent further thrombosis and neurologic deficit progression in this patient. We recommend reducing the size of the BTS stump during definitive repair.

Origin of the circumflex coronary artery (Cx) from the pulmonary arteries is an extremely rare anomaly. We describe a two-month-old female patient with anomalous origin of the Cx from the right pulmonary artery associated with coarctation of the aorta. Reimplantation of the anomalous Cx to the aorta and coarctation repair were performed. There were no postoperative complications, and the patient was discharged in satisfactory condition.