Validation of the Vasoactive-Ventilation-Renal Score for Neonatal Heart Surgery.

OBJECTIVES:  We aimed to validate the vasoactive-ventilation-renal (VVR) score and to compare it with other indices as a predictor of outcome in neonates recovering from surgery for critical congenital heart disease. We also sought to determine the optimal time at which the VVR score should be measured. METHODS: We retrospectively reviewed neonates recovering from cardiac surgery between July 2017 and June 2020. The VVR score was calculated at admission, 24, 48, and 72 hours postoperatively. Max values, defined as the highest of the four scores, were also recorded. The main end result of interest was a composite outcome which included prolonged intensive care unit stay and mortality. Receiver operating characteristic curves were generated, and areas under the curve with 95% confidence intervals were calculated for all time points. Multivariable logistic regression modeling was also performed. RESULTS: We reviewed 73 neonates and 21 of them showed composite outcomes. The area under the curve value for VVR score as a predictor of composite outcome was greatest at postoperative 72-hour max (AUC= 0.967; 95% confidence interval, (0.927-1). On multivariable regression analysis, the VVR max 72 hours remained a strong independent predictor of prolonged ICU stay and mortality (odds ratio, 1.452; 95% confidence interval, 1.036-2.035). CONCLUSIONS: We validated the utility of the VVR score in neonatal cardiac surgery for critical congenital heart disease. The VVR follow-up in postoperative 72 hours is superior to other indices and especially the maximum VVR value is a potentially powerful clinical tool to predict ICU stay and mortality.

The Ideal Timing of Bedside Surgical Ligation of Patent Ductus Arteriosus in Premature Babies Less Than 30 Gestational Weeks.

OBJECTIVE: The aim of our study is to determine the relationship between exposure to hemodynamically significant patent ductus arteriosus and morbidities in premature babies, the optimal number of pharmacologic treatment cycles, and ideal ductus ligation timing. MATERIALS AND METHODS: The study was a retrospective single-center study conducted in a 3-year period between July 2017 and June 2020. Premature babies, born ≤30 weeks of gestation and transferred to our unit for bedside ductus ligation, were included in the study. The subjects were divided into 2 groups; Group A consisted of the patients who received ≥3 pharmacologic treatment cycles, and group B consisted of the patients who received ≤2 cycles. The groups were compared according to preoperative and postoperative features. The main outcome of the study was the presence of severe bronchopulmonary dysplasia. The secondary outcomes were specified as the length of stay in the neonatal intensive care unit and the duration of invasive mechanical ventilation (MV). RESULTS: The study group consisted of 24 patients. There were 10 patients in group A and 14 patients in group B. The mean gestational week and the mean birthweight were found to be 26,7 ± 2.2 weeks and 928 ± 190 g, respectively. The incidence of severe bronchopulmonary dysplasia was significantly higher in group A (70% vs. 14.3%; P = .019). Post-ligation invasive MV, duration, and length of stay in the intensive care unit were found to be significantly longer in group A. None of the patients had hemodynamic disturbances or complications during and after the operation. CONCLUSIONS: Bedside surgical ductus ligation is a safe procedure. Prolonging pharmacologic treatment in order to avoid surgery increases the risk of severe bronchopulmonary dysplasia and prolongs hospital stay.

Ninety-two days of extracorporeal membrane oxygenation support following congenital heart surgery.

We report on a case involving a 10-month-old infant who received prolonged ECMO therapy following cardiac surgery for multiple ventricular septal defects (VSD). The patient was successfully weaned from 92 days of ECMO support without any long-term deficits.

Bedside PDA ligation in premature infants less than 28 weeks and 1000 grams.

BACKGROUND: PDA(Patent ductus arteriosus) is a common and clinically important condition which is presented with a number of hemodynamic and respiratory problems such as intraventricular hemorrhage, pulmonary hemorrhage and necrotizing enterocolitis due to increased pulmonary blood flow and stealing from systemic circulation. The incidence of PDA among the infants that were born before the 28th gestational week is as high as 70 %; and spontaneous closure rates in very-low-birth-weight premature neonates(VLBWPN) is around 34 %. The onset, duration, and repeat number of consecutive courses of the prostaglandin synthesis inhibitor medication for PDA closure are still issues of debate. Bed-side PDA closure is a safe surgical procedure in both mature and premature babies. Here we aim to retrospectively present our 26 cases which were less than 28 weeks and 1000 grams that underwent bed-side PDA ligation. METHODS: This retrospective study included 26 VLBWPN with PDA that underwent bed-side ligation between 2012 and 2015. Babies were born before the 28th gestational week (23-27 weeks) and less than 1000 grams (489-970 gr). Of the 26, 15 were female and 11 were male. Indomethacin was administered to all of the cases as the medical closing agent. The medication was stopped due to unwanted effects in 6 cases. All of the patients took medical treatment before surgery. RESULTS: No surgical mortality occurred during our study. One case of pneumothorax was recorded as late surgical complication. Five of the 26 patients were lost, and the most common cause of mortality was sepsis (in 3 cases). The remaining 21 cases were discharged on days 86-238. The follow-up periods of the patients were 2 moths - 3 years. The most frequent problems encountered after discharge was chronic lung problems. CONCLUSIONS: Bed side PDA ligation surgery in the ICU is a safe method for VLBWPN with clinically significant PDA.

Double Switch Procedure and Surgical Alternatives for the Treatment of Congenitally Corrected Transposition of the Great Arteries.

BACKGROUND: We present our experience with the double switch operation in sixteen patients with congenitally corrected transposition of the great arteries. METHODS: We enrolled 16 patients with congenitally corrected transposition of the great arteries operated by a single surgeon between 1995 and 2015. The mean age was 25 months (range 4 to 72 months) and the mean body weight was 8.9 kg (range 4.3 to 19 kg) at the time of operation. RESULTS: We encountered seven patients with moderate to severe tricuspid regurgitation, five of which had Ebstein anomaly. We performed a combination of atrial and arterial switch procedures in 11 cases, one of which had a concomitant coarctation of the aorta that was repaired along with the double switch procedure. Atrial switch and the Rastelli procedures were performed in three cases with concomitant pulmonary stenosis. A combination of arterial switch, Hemi-Mustard procedure, and bidirectional cavopulmonary anastomosis was performed in two cases. During a mean follow-up period of 67 months (range three months to 18 years), we encountered one early postoperative mortality related to intracerebral bleeding. All but one of the patients are now in NYHA class I-II. CONCLUSIONS: Congenitally corrected transposition of the great arteries is a rare congenital cardiac anomaly in which the results of the anatomical repair with double switch operation appear to be superior to that achieved by a physiological repair.

Mid-Term Results of Congenital Supravalvar Mitral Ring Resection.

BACKGROUND: Supravalvar mitral ring is a rare congenital anomaly leading to left ventricle inflow obstruction. We present our surgical experience and mid-term results in patients operated for supravalvar mitral ring and associated congenital heart defects. MATERIALS AND METHODS: A retrospective analysis was performed in order to evaluate the cases surgically treated with the diagnosis of supravalvar mitral ring between 2001 and 2014. Ten patients were identified, seven of whom had accompanying congenital heart defects. Median age at the operation was 4.5 years. RESULTS: Ventricular septal defects were encountered in half of the cases. Two of the patients had mitral annular hypoplasia, another two had the components of Shone's complex. The mean preoperative gradient across the supravalvar mitral ring decreased from 14.1 ± 4.2 mmHg to 4.6 ± 2.2 mmHg. All patients are alive and remain in a good clinical condition after a mean follow-up of five years. CONCLUSION: Supravalvar mitral ring is a surgically treatable cause of left ventricular inflow obstruction. Although residual gradients may be encountered in patients with mitral annular hypoplasia, surgical resection of the ring is encouraged.

Modified Single Versus Double-Patch Technique for the Repair of Complete Atrioventricular Septal Defect.

OBJECTIVES: We present our single center results of two commonly used surgical techniques, the modified single-patch and double-patch technique for the repair of the complete atrioventricular septal defect. METHODS: We enrolled 49 patients with complete atrioventricular septal defect who were operated by a single surgeon between 2004 and 2014. The modified single-patch technique was performed in 32 cases (group S), whereas the defect was repaired with the double-patch technique in 17 patients (group D). RESULTS: The mean age at the time of operation was 7.7 ± 8.6 (range 1-48) months and 9.9 ± 12.5 (range 1.5-48) months, in groups S and D, respectively. Mean body weight was 6.7 ± 3 (range 3.5-15.5) kilograms and 7.2 ± 3.8 (range 4.3-14.5) kilograms in groups S and D, respectively. Cross-clamp and cardiopulmonary bypass times were shorter in the modified single-patch group (65.6 ± 16.3 vs. 98.7 ± 19.8 minutes, p = 0.0001; 88.9 ± 23.3 vs. 128.9 ± 28.0 minutes, p = 0.0001). Postoperative atrioventricular valve function was improved in both groups. Mean follow-up period was 4.4 ± 3.2 (range 1.2-9.9) years. One patient in each group underwent reoperation for left atrioventricular valve insufficiency. We encountered two early and one late mortalities in modified single-patch group. One early mortality was experienced in the unmodified group. CONCLUSIONS: The modified single-patch and two-patch techniques have comparable results; however, the modified single-patch technique is performed with significantly shorter cross-clamp and cardiopulmonary bypass times, therefore we have adopted this technique on a routine basis for the treatment of the complete atrioventricular septal defect in our institute.

Tissue Doppler imaging in rheumatic carditis.

OBJECTIVE: Our study was undertaken to assess cardiac functions by tissue Doppler echocardiography in patient with primary episode of rheumatic carditis. METHODS: We divided 82 patients with rheumatic carditis were divided in two groups; 50 patients with mild and 32 patients with mitral regurgitation of grade two or more. A control group consisted of 30 healthy children free of any disease. All children underwent conventional and tissue Doppler echocardiography initially and at the time of the follow-up examination. RESULTS: Myocardial systolic wave velocity of the mitral annulus was significantly higher in patients with mitral regurgitation of grade two or more when compared to the control group, but was not different between patients with mild mitral regurgitation and healthy subjects at the time of the initial attack. Myocardial precontraction time, myocardial contraction time, and the ratio of myocardial precontraction and contraction times were significantly prolonged, and the systolic myocardial velocity of the mitral annulus was significantly decreased in patients with mitral regurgitation of grade two or more at the time of the follow-up examination. The myocardial systolic wave velocity was significantly lower, and myocardial precontraction time, myocardial contraction time, and the ratio of the precontraction and contraction times, were significantly longer or greater between patients with grade two or more mitral regurgitation and the control group at follow-up examination. CONCLUSION: We detected subclinical systolic dysfunction of the left ventricle in children with a primary episode of rheumatic carditis due to ongoing ventricular volume overload. Tissue Doppler imaging provides a quantifiable indicator useful for cardiac monitoring of disease during the period of follow up.

Inhaled iloprost in preterm infants with severe respiratory distress syndrome and pulmonary hypertension.

OBJECTIVE: Many vasodilator drugs, including inhaled iloprost, are used to treat insufficient pulmonary vasodilatation, which is the main issue in pulmonary hypertension in newborns. STUDY DESIGN: The safety and efficacy of inhaled iloprost for the treatment of pulmonary hypertension were evaluated retrospectively in 15 preterm infants diagnosed with respiratory distress syndrome and pulmonary hypertension. RESULTS: The infants were unresponsive to surfactant and conventional mechanical ventilation and thus were treated with inhaled iloprost. Oxygenation parameters and hypoxemia improved rapidly after treatment. There was no decline in systemic blood pressure, no need for increased doses of vasopressor, and no side effects during treatment. One patient died of sepsis during treatment. CONCLUSION: In the treatment of severely sick premature babies with pulmonary hypertension, inhaled iloprost has high tolerability and a low incidence of systemic side effects. Based on the benefits of inhaled iloprost in preterm infants with pulmonary hypertension in this case series, further studies are required to evaluate its efficacy and safety in the preterm population.

Transthoracic echocardiographic guidance during transcatheter closure of atrial septal defects in children and adults.

The purpose of this study was to evaluate the safety and efficacy of transcatheter atrial septal defect (ASD) closure guided by transthoracic echocardiography (TTE). Since 2004, ASD closure was performed successfully in total 337 patients. Transthoracic echocardiography guidance was used in 206 patients (61.1%) (group 1). Closure was guided by transesophageal echocardiography under general anesthesia in patients with poor transthoracic acoustic windows, defects with aneurysmatic septum and/or multiple defects in 131 patients (38.9%) (group 2). The median age (9 vs. 16 years, P < 0.001), mean defect diameter (14.9 ± 4 vs. 17.2 ± 5 mm, P < 0.001), ratio of complex atrial septal defect (14 vs. 34%, P = 0.01), the median balloon stretch dimensions (21 vs. 18.7 mm, P = 0.003) and the median device diameters (22 vs. 19 mm, P < 0.001) were significantly greater in group 2 compared to group 1. Both the median procedure time and the median fluoroscopy time was significantly shorter in group 1 (60 vs. 75; and 13 vs. 16.5 min; P < 0.0001 and P < 0.0001, respectively). The incidence of residual shunt did not differ significantly in two groups during follow up. Transthoracic echocardiography guidance during transcatheter ASD closure is safe and effective in children and in many adults. Even complex ASDs could be closed with TTE in patients with good acoustic windows. Performing the procedure under TTE guidance significantly reduces procedure time and also provides increased patient's comfort.

Inhaled iloprost in the treatment of pulmonary hypertension in very low birth weight infants: a report of two cases.

We treated 2 very low birth weight (VLBW) infants with respiratory distress syndrome suffering from refractory hypoxic respiratory failure complicated with severe pulmonary hypertension with inhaled iloprost. The first infant was an 800 gram male and the second case was a 920 gram female. Echocardiography revealed a right to left shunt through patent duct in the first case; suprasystemic pulmonary arterial pressure was estimated by using tricuspid regurgitation of moderate severity in the second case. Inhaled iloprost was started in those infants when conventional therapies including the administration of exogenous surfactant and high-frequency oscillatory ventilation failed. After the commencement of therapy, the clinical condition of the infants improved dramatically. Pulmonary arterial pressure returned to normal levels within five days. We suggest that inhaled iloprost may be helpful by improving oxygenation and reducing the need for aggressive mechanical ventilation in some cases of severe hypoxaemic respiratory failure in VLBW infants.

Successful transcatheter closure of a Fontan fenestration with a bioabsorbable Biostar occluder.

We report the successful closure of an extracardiac Fontan fenestration with a bio-absorbable device, which may be refenestrated by a transcatheter route when needed, in a 10-year-old boy. The patient presented with cyanosis two years after an extracardiac Fontan operation. Echocardiography revealed a moderate shunt from the Fontan circulation into the systemic circulation with a mean pressure gradient of 3-4 mmHg. Treadmill testing revealed a significant decrease in oxygen saturation (down to the low 50's from a baseline level of 80-85%). Cardiac catheterization revealed normal pressure in the Fontan circuit. A temporary balloon occlusion test showed that the defect was suitable for permanent occlusion. The fenestration was then occluded by a bio-absorbable Biostar (NMT medical, Boston, USA) atrial septal occluder device. The oxygen saturation on room air increased up to 95% after closure.

Transcatheter device closure of a residual postmyocardial infarction ventricular septal defect.

Postmyocardial infarction ventricular septal defect (VSD) carries a high mortality and, even after successful surgery, residual defect is common. A 75-year-old woman was admitted with the diagnosis of hyperacute anterior myocardial infarction. Primary percutaneous intervention was performed by stenting of a totally obstructed segment in the proximal left anterior descending artery. The patient's condition deteriorated on the second postprocedural day with a 3/6 pansystolic murmur at the mesocardium. Echocardiography revealed an apical anteroseptal VSD and moderate pulmonary hypertension. She underwent surgical VSD closure with a Gore-Tex patch and coronary artery bypass grafting to the left anterior descending and circumflex arteries. The patient's condition continued to be unstable due to septicemia and hemodynamically significant residual VSD. After medical management of septicemia, the residual defect was successfully closed using a 10-mm Cardio-O-Fix septal occluder under fluoroscopic and transesophageal echocardiographic guidance. The clinical condition of the patient was then stabilized and there was no significant residual shunt on echocardiography on the third postprocedural day.

Atrial septal stenting to increase interatrial shunting in cyanotic congenital heart diseases: a report of two cases.

Aiming to increase mixing at the atrial level, atrial septal stenting was performed in two pediatric cases with cyanotic congenital cardiac diseases. The first case was a 3-month-old male infant with transposition of the great arteries. The second case was an 18-month-old male infant with increased central venous pressure due to postoperative right ventricular outflow tract obstruction. Premounted bare stents of 8 mm in diameter were used in both cases. The length of the stent was 20 mm in the first case and 30 mm in the latter. The procedure was accomplished without any complications. In the first case, oxygen saturation increased approximately 20-25% with no significant interatrial gradient. In the latter, central venous pressure decreased from 16 to 8 mmHg immediately after the procedure. The patient was weaned from the ventilator on the second day and discharged from intensive care unit on the fifth day. Follow-up echocardiograms of both patients showed patent stents with good position relative to the atrial septum. Stenting of the atrial septum seems to be a safe and effective method to create a reliable, nonrestrictive interatrial communication.

[Our medium-term results with aortic balloon valvuloplasty and factors affecting development of aortic regurgitation]. / Aort balon valvüloplastisi'nde orta-dönem sonuçlarimiz ve aort yetersizligi gelisimini etkileyen faktörler.

OBJECTIVE: The objective of this study was to assess medium-term results of aortic balloon valvuloplasty (ABV) in congenital aortic stenosis and to determine factors affecting development of aortic regurgitation (AR). METHODS: Between September 2003 and January 2010, 114 ABV procedures performed on 97 patients analyzed retrospectively. Patients were evaluated in terms of transvalvular gradients before and after procedure, development of new aortic insufficiency or increase in the current insufficiency and factors affecting the procedural success and development of aortic insufficiency. In addition, follow up results were also evaluated in terms of restenosis and increase or decrease of aortic regurgitation. Student's t-test, ANOVA for repeated measurements, linear discriminant and Kaplan-Meier survival analyses were used for statistical analysis. RESULTS: The mean age was 6.63±6.33 year (2 days-21 years). Mean follow-up was 32.6±25.1 months (1 to 75 months). Thirty-seven patients were infant (<1 year) and 18 of them were newborn. The peak systolic pressure gradient decreased from 77.2±24.9 mmHg to 28.5±12.9 mmHg (p=0.0001). Eighty-two procedures were optimal, 29 suboptimal and 3 were unsuccessful. AR developed in totally 53 patients, 16 of them were significant degree. Balloon /annulus ratio and the percentage of reduction in gradient was meaningfully higher in patients with significant aortic regurgitation (p=0.02 and p=0.03, respectively). Infants show more significant AR (9/37, 24%) in comparison with bigger patients (7/77, 9%) (p=0.02). Four patients died after procedure at intensive care unit, three of them were newborn. CONCLUSION: ABV is safe and effective palliative method for the treatment of congenital aortic stenosis. AR, which was the most common complication, incidence was increased in infancy and was related with high balloon/annulus ratio and high reduction in gradient.

Early Transient Neonatal Cyanosis Related to Interatrial Right-to-Left Shunting at an Altitude of 1890 Meters: A Report of Five Cases.

BACKGROUND: We report five term neonates born at an altitude of 1890 meters with transient early neonatal cyanosis due to right-to-left shunting at atrial level through patent foramen ovale. CASE PRESENTATION: The five neonates with no clinical sign or symptom other than marked cyanosis were examined in two neonatal units of Erzurum city. Hematologic and radiologic examinations were normal. Partial oxygen pressure (PO(2)) in the arterial blood samples was lower than 45 mmHg in all of the patients, and did not increase more than 15 mmHg in any of the patients after inhalation of 100% oxygen. Echocardiography revealed normal intracardiac structure. The right-to-left interatrial shunt at diastole was detected through a patent foramen ovale in all of these infants. By only observation with no treatment, diastolic right-to-left shunt disappeared in 40.15±9.52 hours. Oxygen saturation was increased from 69.80±9.55 percent to 90.40±8.80 percent. The patients were discharged from the hospital at 5.6±0.4 days of life. Follow up for 6 months revealed no clinical problem in any of the cases. CONCLUSION: Transient cyanosis can be seen in the very early neonatal period because of interatrial right-to-left shunting in some healthy term neonates born at an altitude of 1890 meters. Decreased right atrial compliance due to relative hypoxia at that altitude can be speculated to be the causative mechanism.

Evaluation of cardiac function in healthy children native to 1890 metres.

OBJECTIVE: The aim of the study is, by comparing cardiac parameters between children native to 1890 metres with children living at sea level, to find out whether there is any impairment in cardiac function related to that altitude. METHODS: Electrocardiographic, conventional, and tissue Doppler echocardiographic parameters were compared in 42 healthy children native to 1890 metres, and in 21 healthy age and gender matched children living at sea level. Plasma haemoglobin level and oxygen saturation measured by pulse oxymeter were also obtained from all patients. RESULTS: Haemoglobin levels were higher, and oxygen saturation levels were lower in children native to 1890 metres. Conventional echocardiographic parameters and mitral annular myocardial parameters were all similar between children native to 1890 metres and children living at sea level. Tricuspid lateral annular early diastolic velocity and the ratio of early-to-late diastolic velocity were significantly lower and tricuspid lateral annular izovolumetric relaxation time was significantly higher in children native to 1890 metres than children living at sea level. CONCLUSION: Children living at 1890 metres of altitude predispose to asymptomatic right ventricular diastolic dysfunction or otherwise they remain as healthy children.

The effect of low-carbohydrate diet on left ventricular diastolic function in obese children.

BACKGROUND: This study was conducted to evaluate left ventricle (LV) functions using conventional and tissue Doppler imaging in childhood obesity and to identify the effects of diet on LV diastolic functions. METHODS: Conventional and tissue Doppler echocardiographic measurements were compared in 34 obese children and 24 age- and gender-matched lean controls. Fasting plasma glucose, insulin and homeostatic model assessment of insulin resistance levels were also obtained. Thirty-one of the obese children were subjected to a low-carbohydrate diet and their follow-up measurements were obtained after 6 months. RESULTS: Left atrial diameter, LV mass and LV mass index were higher in obese children than in lean controls. Lateral mitral myocardial early diastolic (E(m)) and peak E(m)/myocardial late diastolic (A(m)) were lower, and mitral E/E(m) and lateral mitral myocardial isovolumetric relaxation time were higher in obese subjects than in lean controls. Insulin and homeostatic model assessment of insulin resistance levels were higher in obese patients and decreased significantly after diet. After diet therapy, lateral mitral E(m) and peak E(m)/A(m), were increased, mitral E/E(m) and myocardial isovolumetric relaxation time were decreased. CONCLUSIONS: Obesity predisposes children to increased preload reserve, left ventricular subclinical diastolic dysfunction and deterioration in diastolic filling. Weight reduction with a low-carbohydrate diet seems to be associated with significant improvement in LV diastolic function and a decrease in diastolic filling, as well as causing reversal in insulin resistance seen in obese children.

Anomalous origin of one pulmonary artery branch from the ascending aorta: experience of our center.

OBJECTIVES: Anomalous origin of one pulmonary artery branch from the aorta in the presence of separate aortic and pulmonary valves is a rare but important entity necessitating early diagnosis and surgery to prevent irreversible vascular pulmonary disease. We evaluated our experience with seven infants having this anomaly. STUDY DESIGN: Between December 2003 and 2009, a total of seven infants (2 girls, 5 boys, age range 4 days to 84 days) were diagnosed as having anomalous origin of one pulmonary artery branch from the aorta. Clinical records were reviewed for clinical features, operative procedures, and postoperative follow-up. RESULTS: Common symptoms were dyspnea, tachypnea, and poor feeding. All the cases were diagnosed by echocardiography. The right pulmonary artery in six cases and the left pulmonary artery in one case originated from the ascending aorta. In addition, three patients had patent ductus arteriosus (PDA), five patients had patent foramen ovale, and one patient had interruption of the aortic arch and aortopulmonary window. All patients underwent surgical re-implantation of the anomalous pulmonary artery branch to the pulmonary trunk. Associated surgical procedures included PDA ligation in three patients, and total repair of interrupted aortic arch and aortopulmonary window in one patient. There were no in-hospital deaths. Two patients had prolonged intubation (10 and 16 days). All patients were discharged in good clinical condition. During a follow-up period ranging from two months to six years, only one patient developed stenosis at the site of anastomosis. CONCLUSION: Prompt diagnosis at infancy, improved surgical technique, and good patient care decrease mortality and morbidity associated with anomalous origin of the pulmonary artery from the aorta.

[Arterial tortuosity syndrome in two cases]. / Iki olguda kivrimli arter (tortuosity) sendromu.

Arterial tortuosity syndrome (ATS) is a rare autosomal recessive connective tissue disease characterized by elongation, tortuosity, aneurysmatic formation, and stenotic lesions in large and medium-size arteries. We present two cases of ATS diagnosed during cardiac examination for murmurs. The first was an 11-year-old boy who had an atypical facial appearance and hyperelasticity. He had a prior operation for inguinal hernia. Echocardiography showed aneurysmatic dilatation in the main pulmonary artery and peripheral stenotic lesions. Angiography and computed tomography angiography confirmed aneurysmatic formation in the main pulmonary artery and multiple stenotic lesions in peripheral arteries and showed elongation and tortuosity of the major branches of the aorta. Surgical reconstruction of the pulmonary arterial system was performed. The second was a 3-month-old girl with an atypical facial appearance, hyperelasticity, and marked hypotonia. The aortic arch could not be visualized during echocardiography. Angiographic examination showed mild bilateral stenosis of distal pulmonary arteries, elongation and tortuosity of the aortic arch and its main branches.