Nonconfluent Pulmonary Arteries Research Articles

Bilateral ductal stenting in an infant with pulmonary atresia and ventricular septal defect, non-confluent pulmonary arteries, and bilateral arterial duct.

Pulmonary atresia with ventricular septal defect, non-confluent pulmonary arteries, and bilateral arterial duct is a rare and complex CHD. Physiologic ductal closure may lead to life-threatening hypoxia. We present a case of successful bilateral ductal stenting as a bridge to further lower-risk surgical repair.

Pulmonary artery coarctation repair in univentricular heart: indications and optimal timing.

To analyse surgical outcomes of pulmonary artery coarctation in univentricular hearts, focusing on surgical indications and optimal timing. We retrospectively reviewed 49 patients with pulmonary artery coarctation in univentricular hearts treated at our institution between 1993 and 2022. Twenty-eight patients were diagnosed before first-stage palliation. Of these, 14 underwent systemic-pulmonary shunt only as first-stage palliation (Group 1), and 14 underwent systemic-pulmonary shunt plus surgical pulmonary artery plasty as first-stage palliation (Group 2). Twenty-one patients diagnosed after first-stage palliation underwent surgical pulmonary artery plasty at the time of bidirectional Glenn procedure (Group 3). Follow-up period after initial palliation was 6±8 years. The Fontan procedure was successful in 35 patients (71%) aged 28±26 months (range 18-139). Freedom from interstage death (Group 1, 53%; Group 2, 85%; Group 3, 93%) and interstage reintervention (Group 1, 50%; Group 2, 75%; Group 3, 73%) rates were significantly lower in Group 1 (p = 0.01). Five and four patients in Group 1 and Group 3, respectively, needed additional shunts before the bidirectional Glenn procedure. In Group 1, one patient with a non-confluent pulmonary artery achieved hemi-lung Fontan circulation. In Group 2, one patient suffering with a non-confluent pulmonary artery could not achieve Fontan circulation, whereas another patient with pulmonary venous obstruction achieved hemi-lung Fontan circulation. Surgical pulmonary artery plasty performed at first-stage palliation improved outcomes of pulmonary artery coarctation in univentricular hearts, particularly when pulmonary artery coarctation had already progressed during the neonatal period or early infancy.

Congenital Non-Confluent Pulmonary Artery Sourced from Bilateral Arterial Ducts: A Rare Anomaly.

This study aimed to identify the influence of the non-confluent pulmonary artery originating from the bilateral arterial ducts (AD) on the outcomes of the Fontan circulation. We retrospectively reviewed the records of nine patients with bilateral AD and a non-confluent pulmonary artery in a single ventricle at our institution between 1993 and 2023. Three patients showed maintained AD or underwent a systemic-pulmonary shunt for stenotic AD, followed by the Glenn procedure. Four patients underwent systemic-pulmonary shunt with angioplasty as the first palliation, followed by the Glenn procedure. Two patients underwent systemic-pulmonary shunt or AD stenting as the first palliation and systemic-pulmonary shunt with angioplasty as the second palliation. There were no cases of interstage mortality. Pulmonary arteries grew and achieved a good balance (pre-Fontan pulmonary artery index [PAI], 164 ± 27 mm2/m2; right/left PAI ratio, 1.06 ± 0.23). All patients underwent the Fontan procedure (median, 3.5years; range, 2.3-6.4years) and were followed up for 7.8years (range, 0.1-16.4years) after the procedure. One patient required hospitalization for heart failure at 1.8years, and three patients required catheter intervention for pulmonary stenosis within 2months after the Fontan procedure. Non-confluent pulmonary arteries originating from the bilateral AD do not preclude Fontan completion and good Fontan outcomes by restoring balance to pulmonary blood flow. Due to the limited sample size in this study, additional research is imperative to delve deeper into our findings and enhance understanding of the most effective surgical approach for this disease.

Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). Data about the outcomes in developing countries is scarce. We therefore conducted a retrospective study to assess survival rates and mortality risks of 90 children with PA/VSD at Siriraj Hospital, Thailand during 2005–2016. Patients with single ventricle were excluded. Survival and mortality risks were analyzed at the end of 2018. The median age of diagnosis was 0.5 (0–13.8) years. The patients’ PAs were categorized into four groups: 1) PA/VSD with confluent PAs (n = 40), 2) PA/VSD with confluent PAs and MAPCAs (n = 21), 3) PA/VSD with non-confluent PAs and MAPCAs (n = 12), and 4) PA/VSD with small native PAs and MAPCAs (n = 17). Of the 88 patients who underwent operations, 32 patients had complete repair at 8.4 ± 4.6 years old. During the follow-up [median time of 5.7 years (7 days-13.6 years)], 17 patients (18.9%) died. The survival rates at 1, 5, and 10 years of age were 95%, 83.7%, and 79.6%, respectively. Significant mortality risks were the presence of associated anomalies and non-confluent PAs.

Intraoperative Flow Study Predicted the Postoperative Pulmonary Artery Pressure in the Bidirectional Glenn Operation.

The aim of this study was to evaluate the predictability of postoperative pulmonary artery pressure (PAP) using intraoperative flow study in patients undergoing bidirectional Glenn operation. Patients who underwent Glenn operation under cardiopulmonary bypass (CPB) were included in the study. During the operation, after the completion of additional procedures under CPB, an intraoperative flow study was performed prior to Glenn anastomosis. After the completion of bidirectional Glenn, the patient was separated from the CPB and PAP was measured. The relationship between this pressure and flow study measurement was analyzed. Nine patients who underwent bidirectional Glenn operation with additional procedures under CPB between July 2018 and January 2019 were included in the study. The median PAP was 9 mm Hg (interquartile range [IQR]: 7-10 mm Hg) in the flow study and 10 mm Hg (IQR: 8-11 mm Hg) after CPB, and the median difference between these pressures was 1 mm Hg (IQR: 1-3 mm Hg). There was a strong correlation between these two measurements (r = 0.732; P = .025). The results of this study show that PAP after the Glenn procedure can be estimated using an intraoperative flow study. We believe that this method may be useful in intraoperative decision-making for Glenn operation in single ventricular patients who require extensive pulmonary artery (PA) reconstruction due to limited PA development, branch PA stenosis, or nonconfluent PAs. Also, this method can be used as a sort of intraoperative pulmonary resistance reversibility study in patients with high preoperative pulmonary vascular resistance due to surgically correctable pulmonary venous hypertension.

Extensibility of autologous pericardium roll conduit in non-confluent pulmonary artery: a case report

BackgroundThere is great discussion about non-confluent pulmonary artery (PA) reconstruction, and several materials have been used. Autologous pericardium is considered feasible for infectious resistance, autoimmune response, extensibility, and growth potential.Case presentationThe patient was born at 39 weeks (body mass = 2550 g). He was diagnosed with tetralogy of Fallot, pulmonary atresia, non-confluent PA, and bilateral patent ductus arteriosus. Right and left Blalock-Taussig shunts with patent ductus arteriosus ligations were placed on day 27 and 3 months, respectively. At 19 months (8.8 kg), definitive repair was performed with tricuspid valved conduit concurrent with PA reconstruction using an autologous pericardium roll conduit. The autologous pericardium was treated with glutaraldehyde (autologous pericardium fixed with 0.4% glutaraldehyde for 7 min and rolled as conduit – 12 mm in diameter and 30 mm in length). Following an incision on the visceral side of the PAs before the 1st branch, the autologous pericardial roll conduit was anastomosed. Follow-up angiographies on postoperative months 9 and 57 demonstrated that the PA, including the autologous pericardium roll conduit, had spontaneously enlarged.ConclusionParticularly for non-confluent PA, the patients require increased pulmonary beds at an early age because of hypoplastic PA. While size mismatch between the graft and native PA develops as the child grows, size-adjustable extensibility of the PA graft should be noted.

Total Correction of Tetralogy of Fallot in the First 60 Days of Life in Symptomatic Infants: Is It The Gold Standard?

The timing of surgical repair of tetralogy of Fallot (TOF) is a key to alleviate complications and for long-term survival. Total correction was usually performed at the age of 6 months or older under the notion of decreasing the surgical risk. However, avoiding palliation with an aortopulmonary shunt and early correction of systemic hypoxia appear to be of more benefit than the inborn surgical risk in low body weight patients. Our objective was to assess early/midterm survival and operative complications and to analyze patients, surgical techniques, and morphological risk factors to determine their effects on outcomes. We retrospectively reviewed 152 patients with TOF who were ≤60 days of age when they underwent total correction of TOF. All patients had either duct-dependent pulmonary blood flow or arterial blood oxygen saturation less than 65% on room air requiring urgent surgical correction. Exclusion criteria included TOF with pulmonary atresia, TOF with nonconfluent pulmonary arteries, TOF with multiple aortopulmonary collateral arteries, and associated complete atrioventricular septal defects. The mean age at repair was 34 ± 19 days, and the mean weight was 3.8 ± 0.9 kg. Before surgery, 96 patients received an infusion of prostaglandin, 45 were mechanically ventilated, and 32 required inotropic support. Right ventricular outflow tract obstruction was managed with a transannular patch in 112 patients, and all the others had a main pulmonary artery patch. Cardiopulmonary bypass (CPB) with moderate hypothermia was the standard, and the CPB time averaged 48 ± 21 minutes. The postoperative intensive care unit stay was 5.7 ± 6 days, with 2.8 ± 4 days of mechanical ventilation. Early mortality was 4.6% (7 of 152), and actuarial survival rates were 95% at 1 year and 92% at 5 years. Univariable and multivariable analyses of the patients' demographics, anatomical characteristics, and operative techniques revealed the presence of small pulmonary arteries and low body weight to be the only independent risk factors for death. Early total correction of TOF during the first 60 days of life can be performed with low mortality and good intermediate-term survival and, from our point of view, "should be the gold standard for TOFs."

53. Bilateral ductal stenting for nonconfluent pulmonary arteries in a newborn

Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Limited data available for bilateral PDA stenting. Bilateral PDA stenting in nonconfluent pulmonary arteries is challenging procedure but can be considered as an option in the management of complex conditions like this. 12 days old Preterm (36 weeks gestation) male baby with birth weight of 2.6 kg developed respiratory distress with severe cyanosis and desaturation upto 50%. Baby was intubated and started on Prostaglandin 0.05 mic/kg/mt. His saturation improved to 80%. Echocardiogram showed complex cyanotic heart disease, Situs ambiguous, dextrocardia, complete unbalanced AV septal defect, pulmonary atresia , nonconfluent small branch pulmonary arteries supplied by the bilateral patent ductus arteriosus (PDA) from right aortic arch and all four pulmonary veins form a confluence and drain into superior vena cava(SVC )through vertical vein with no obstruction. Baby was taken up for PDA stenting. descending aortogram showed right aortic arch with vertical tortuous duct to right pulmonary artery (RPA) and another short duct with acute angle from left subclavian artery to left pulmonary artery (LPA ). Both ducti stented with coronary stents. Vertical vein angiogram showed both lungs drain to a confluence and then to SVC via ascending vertical vein with no obstruction. After stenting lung perfusion improved and the baby was stable and maintained 80% saturation on room air. Bilateral PDA dependent pulmonary circulation with right and left pulmonary artery discontinuity is very rare. Our case is unique with Heterotaxy, TAPVC, Dextrocardia and double ducti. Eventhough bilateral ductal stenting is technically challenging it is successful through femoral artery approach.

Efficacy of the 'intrapulmonary-artery septation' surgical approach for Fontan candidates with unilateral pulmonary arterial hypoplasia†.

The bilaterally unbalanced development of pulmonary arteries (PAs), as a result of unilateral pulmonary arterial hypoplasia (PAhypo) makes patients either ineligible for the Fontan operation or candidates for the one-lung Fontan operation. In the present study, we examined the efficacy of intrapulmonary-artery septation (IPAS), a technique we reported in 2007 in which a septation is constructed within the central PA, in patients with unilateral PAhypo. Sixteen patients with unilateral PAhypo and an affected PA index of ≤60 mm(2)/m(2), including non-confluent PA (NCPA), underwent IPAS between January 2000 and March 2012; patients with pulmonary venous obstruction were excluded from this study. We compared the affected PA index values before and after IPAS and after the Fontan operation as well as the bilateral pulmonary blood flow ratio using pulmonary scintigraphy. The post-Fontan operation values of central venous pressure (CVP), pulmonary vascular resistance (PVR), ventricular end-diastolic pressure (VEDP), cardiac index (CI) and arterial oxygen saturation (SaO2) at the most recent cardiac catheterization, as well as the pre-IPAS and post-Fontan New York Heart Association (NYHA) classification levels were examined. One patient died after IPAS (survival rate, 93.8%). Thirteen (86.7%) of the surviving 15 patients underwent the Fontan operation. The mean PA indexes were 25.5 ± 18.9 mm(2)/m(2) before IPAS and 67.9 ± 34.2 mm(2)/m(2) after IPAS (P = 0.003); the mean PA index was 71.1 ± 50.0 mm(2)/m(2) after the Fontan operation. Restenosis did not occur after the Fontan operation, and the PA diameters were maintained. The mean affected/unaffected lung blood flow ratio was 0.89 ± 0.67. The most recent cardiac catheterization after the Fontan operation indicated the following values: CVP, 11.7 ± 1.8 mmHg; PVR, 1.3 ± 0.4 U m(2); EDP, 5.7 ± 2.0 mmHg; CI, 3.1 ± 0.5 l/min/m(2) and SaO2, 94.9 ± 2.0%. The mean pre-IPAS and post-Fontan NYHA levels were 2.3 ± 0.6 and 1.2 ± 0.4, respectively (P = 0.0002). With IPAS, the affected PA diameters increased significantly, and were maintained after the Fontan operation, and continuity of the native PAs was achieved. IPAS is very effective for patients suffering from otherwise intractable diseases.

Bilateral Ductal Stenting for Discontinuity of the Pulmonary Artery via the Femoral and Carotid Arteries in an Infant.

Bilateral ductal stenting should be performed in cases of discontinuity of the pulmonary branches and pulmonary atresia. Performing this procedure via the carotid artery in small infants can be very difficult and challenging. We present a case of bilateral ductal stenting via both the femoral and carotid arteries in a little child with tetralogy of Fallot with pulmonary atresia and a nonconfluent pulmonary artery and bilateral ductus arteriosus.

Computed tomographic angiography in tetralogy of Fallot

Echocardiography is often inadequate for imaging tetralogy of Fallot, prompting cineangiography. This study prospectively evaluated multidetector computed tomographic angiography for preoperative evaluation of tetralogy of Fallot in 112 consecutive patients. Forty-eight had nonconfluent or hypoplastic pulmonary arteries (mean z-score, -2; range, -11.1-0.13) permitting only palliative or no surgery; 64 had adequate pulmonary artery anatomy (mean z-score, 0.59; range, -2.53-3.4) allowing total repair. The surgical data of 50 patients who underwent total correction were compared with transthoracic echocardiography and multidetector computed tomographic angiography findings. Multidetector computed tomographic angiography tended to reveal unsuspected collaterals and coronary abnormalities besides outlining the right ventricular outflow tract and pulmonary artery branches. The branch pulmonary artery diameter z-score was the most important determinant of surgical strategy, with the worst figures being associated with no surgical options or palliative surgery, and the best figures leading to corrective surgery. The mean radiation dose was 3.45 mSv. Multidetector computed tomographic angiography is a powerful supplement to echocardiography in the preoperative evaluation of tetralogy of Fallot.

Reconstruction of nonconfluent pulmonary artery using the retroaortic innominate vein for a single ventricle

The innominate vein usually courses anterior to the aortic arch, where it joins the right brachiocephalic vein to form the superior caval vein. A retroaortic innominate vein is an uncommon finding in patients with congenital heart disease. We report a patient with a single ventricle, single atrium, pulmonary atresia, nonconfluent pulmonary artery, persistent left superior caval vein, absent inferior caval vein (azygos connection), right aortic arch, and retroaortic innominate vein. His innominate vein took an anomalous course. The right brachiocephalic vein crossed from right to left underneath the aortic arch and formed a left superior caval vein with the left brachiocephalic vein and the azygos vein. We reconstructed the nonconfluent pulmonary artery using the retroaortic innominate vein and then performed a total cavopulmonary shunt when he was 32 months of age. Thereafter, a total cavopulmonary connection was carried out at age 42 months.

Bilateral PDA in a Patient with VSD and Pulmonary Atresia

Patent ductus arteriosus is one of the most common congenital cardiac pathologies, besides patency of ductus may be somewhat vital for various congenital cardiac defects, otherwise death is inevitable. Anatomically, ductus is single and located between the descending aorta and the pulmonary artery. The review of the literature reveals presence of more than one ductus arteriosi in sporadic cases, most commonly associated with aortic arch anomalies. In this report, we present a nine-month-old baby with the diagnosis of ventricular septal defect (VSD), pulmonary atresia (PA), nonconfluent pulmonary arteries, and bilateral patent ductus arteriosi. He underwent a successful pulmonary reconstruction and central-shunt operation with modified aortopulmonary window technique without cardiopulmonary bypass. This is a very rare case with double ductus arteriosi associated with VSD, PA, and nonconfluent pulmonary arteries.

Staged Fontan operation for children with heterotaxy syndrome, bilateral ductus arteriosus, and nonconfluent pulmonary artery

We report two cases of successful Fontan operation in children with heterotaxy syndrome associated with univentricular physiology and absent and nonconfluent central pulmonary arteries with both distal pulmonary arteries directly connected to the ipsilateral ductus arteriosus. After unilateral systemic-pulmonary shunt, the central pulmonary artery was reconstructed with a polytetrafluoroethylene prosthetic graft concomitantly with bidirectional cavopulmonary shunt. Finally, extracardiac total cavopulmonary connection was performed as an off-pump procedure. Children with bilateral ductus arteriosus and a nonconfluent pulmonary artery with univentricular physiology present a particular challenge in regard to completing Fontan operations. Careful attention should be directed at ensuring balanced growth of the bilateral distal pulmonary arteries. When planning reconstruction of the central pulmonary artery with a prosthesis, late reconstruction may be beneficial, as it enables utilization of a larger-caliber graft, obviating the need for replacement during a subsequent Fontan operation.

Long-term results after correction of persistent truncus arteriosus in 83 patients

This study aims to analyse long-term results after correction of truncus arteriosus in all patients operated in one institution over 28 years. Between 1981 and 2009, 83 patients, median age 54 days, underwent repair of truncus arteriosus. Interrupted aortic arch was present in 14 (17%), severe truncal valve insufficiency in 10 (12%) and non-confluent pulmonary arteries in five (6%) patients. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in 80, and other methods in three patients. At the same time, correction of interrupted aortic arch was done in 14 and truncal valve repair in eight patients. Survivors were repeatedly examined echocardiographically for assessment of residual heart lesions. The early mortality was 19 (23%). Out of 35 patients operated between 1981 and 1996, 17 (46%) died, and out of 48 patients operated between 1997 and 2009, two (4%) died. Operation before 1997 (p=0.001) and aortic cross-clamping time >90min (p=0.009) were found to be risk factors of death. Eight (10%) patients died late, a median of 68 days after surgery. Fifty-seven (69%) patients were followed for 10.9 + or - 6.7 years. Three (4%) patients were lost. Twenty-five (30%) patients are alive with their original conduit 7.5 + or - 5.2 years after correction. Twenty-eight patients required 41 re-operations for conduit dysfunction with first replacement at mean 5.8 + or - 4.1 (range 0.1-14.1 years) years after correction. Nine (11%) patients required 12 truncal valve replacements. Eleven (13%) patients required balloon dilatation or stent for conduit obstruction, pulmonary branch stenosis, aortic arch obstruction or stenosis of vena cava. Recent clinical examination was undertaken in 53 (64%) patients and 49 (59% or 77% of early survivors) are in good/very good condition. Truncus arteriosus remains a challenging heart disease. With growing experience, the early mortality decreased to 4%, but numerous re-interventions for conduit obstruction, pulmonary branch stenosis and truncal valve insufficiency are required. Surgery before 1997 and prolonged cross-clamping were risk factors of death. Pulmonary homografts had the best re-intervention-free survival. Statistically, however, the difference between conduits was not significant. Dysplastic valve and truncal valve insufficiency represent risk factors presenting the need for truncal valve replacement.

Staged Approach for Congenitally Corrected Transposition of the Great Arteries Associated with Dextrocardia and Nonconfluent Pulmonary Artery

We completed double switch operation (Senning plus Rastelli procedure; SLL) after staged palliative operations for SLL, dextrocardia, congenitally corrected transposition of the great arteries ventricular septal defect, atrial septal defect, and nonconfluent pulmonary artery. The patient had undergone three preparatory operations including central pulmonary artery reconstruction with autologous pericardium and morphologic right ventricle-pulmonary artery shunt and then double switch operation was performed. During the double switch operation, the previous conduit which was established as right ventricle-pulmonary artery shunt in the third palliation was replaced by a larger conduit. This conduit was used as Rastelli external conduit to avoid coronary artery injury. When systemic venous chamber was reconstructed, suture line pouch technique was used, in which dog ears were made at the sites of superior and inferior vena cava orifices, respectively, to prevent systemic venous return obstruction. Pulmonary venous chamber was reconstructed without any augmentation, which reduced the possibility of postoperative arrhythmia. Systemic and pulmonary venous pathway obstructions were not observed after the operation. Postoperative arrhythmia was not detected.

Creation of an intraatrial tunnel to produce a total cavo-pulmonary connection in a patient with dominant right ventricle, totally anomalous pulmonary venous connection, and pulmonary atresia with non-confluent pulmonary arteries

The surgical strategy for patients having a functionally single ventricle associated with totally anomalous pulmonary venous connection and pulmonary atresia with non-confluent pulmonary artery has yet to be agreed. We created an intraatrial tunnel to produce a total cavo-pulmonary connection in such a patient, also creating a confluence for the pulmonary arteries. By minimizing the use of the GoreTex patch, the patient was able to discontinue the use of warfarin.

Extracardiac Fontan with T-shape conduit in non-confluent pulmonary arteries

A 34 months-old male patient with double inlet right ventricle with nonconfluent pulmonary arteries who underwent successful extracardiac fenestarated Fontan procedure using pre-designed T-shaped PTFE vascular graft after multi-step rehabilitation of the diminutive hilar pulmonary arteries. At first we performed 6 mm confluent pulmonary artery vascular graft implantation with 4 mm BT shunt at patient's 4 weeks old. At 9 months of patient, we upsized the confluent pulmonary arterial graft to 8 mm with bidirectional cavopulmonary connection, and, at 34 months, we performed extracardiac conduit Fontan procedure with pre-designed T-shape conduit including the confluent pulmonary arterial portion at last. Patient shows excellent functional status and development.

Stenting of Bilateral Arterial Ducts in Complex Congenital Heart Disease

Bilateral arterial ducts serving nonconfluent pulmonary arteries is a very rare pattern of pulmonary blood flow in congenital heart disease with pulmonary atresia. In this setting, neonatal ductal closure might result in abrupt pulmonary hypoperfusion and life-threatening systemic hypoxia, thereby indicating emergent surgical palliation or repair. However, percutaneous arterial duct stenting might be an alternative to surgery, especially in high-risk patients. This article reports on two critical neonates with complex heart disease and discontinuous pulmonary arteries dependent on bilateral arterial ducts who successfully underwent transcatheter ductal stenting as first-step palliation toward lower-risk corrective surgery.

Demonstration of Coronary Arteries and Major Cardiac Vascular Structures in Congenital Heart Disease by Cardiac Multidetector Computed Tomography Angiography

Detection of coronary artery is important when considering surgical treatment of a congenital heart disease (CHD) such as tetralogy of Fallot (TF). Cardiac catheterization plays an important supplementary role in the evaluation of patients with CHD. In a few reports, it has been proposed that multidetector computed tomography (MDCT) can be helpful for the visualization of coronary arteries. We sought to demonstrate the feasibility and usefulness of MDCT angiography for anatomical evaluation of coronary arteries in CHD patients with suspected coronary artery anomalies. A total of 10 patients, 9 pediatric and 1 adult, underwent MDCT angiography for the investigation of coronary artery anomalies and mediastinal vascular structures. Seven patients had TF; 5 of these patients were suspected of having coronary artery anomalies and 2 were suspected of having pulmonary artery atresia or a nonconfluent pulmonary artery. The other 3 patients had truncus arteriosus and severe left pulmonary artery stenosis (n = 1), double outlet right ventricle (n = 1), and Kawasaki disease (n = 1) with suspected coronary artery aneurysms. The entire heart, major vascular structures, and coronary artery anomalies were preoperatively scanned in patients with cyanotic heart disease. Examinations were performed by 16-MDCT with 1-mm slice thickness. A breath-holding test was performed in 5 patients. Nonionic iodinated contrast material (2 cc/kg) was administered by a power injector. Major vascular structures and the proximal part of the right and left coronary arteries were visualized successfully in all patients. Mid and/or distal segments of the coronary arteries were visualized in 5 patients with TF. Pulmonary vascular bed findings were also confirmed during surgery in patients with TF and in one patient with truncus arteriosus type I and severe left pulmonary artery stenosis. Kawasaki disease was diagnosed by the presence of aneurysms in one patient. Pulmonary artery atresia was confirmed in one patient and diameter of the pulmonary arteries (4 mm and 4.5 mm) was determined in the other 2 patients by MDCT. The advantage of MDCT for cardiac imaging is the shortened scanning time for imaging the entire heart without long breath-holding times. Selective conventional coronary angiography is invasive and technically difficult in pediatric patients. We suggest that MDCT angiography can be performed as a noninvasive method in patients with CHD for the evaluation of coronary artery anatomy and anomalies and mediastinal vascular structures.