Coronary artery obstructive complications after coronary transfer procedures needed for congenital heart surgery are rare but serious, and it frequently results in death or severe heart failure that requires extracorporeal membrane oxygenation (ECMO) use in the early and late phases of surgery. This complication is particularly crucial in an arterial switch operation for transposition of the great arteries (TGA), which is conducted at the newborn to infant stage when there is a low body-weight. In addition, TGA is commonly associated with various anatomic coronary abnormalities. The following two surgeries are employed to manage this complication: redo of coronary anastomosis, often with autologous patch enlargement (surgical ostial angioplasty: SOAP), and pediatric coronary artery bypass utilizing the internal thoracic artery (PCABS-ITA). Both procedures have advantages and disadvantages compared with the other, but early surgical survival results are equivalent. Based on several database analyses, I currently suggest SOAP as I(C) and PCABS-ITA as IIa(C) for a rescue operation where coronary obstruction is due to mechanical compression, kinking, and/or stretching. For late coronary complications where fibroproliferative obstruction is the main cause, PCABS-ITA as I(C)and SOAP as IIa(C). Furthermore, tight stenosis (>90%) or total obstruction extending into the bifurcation area of the left main trunk favors for PCABS-ITA, and localized left main stenosis of mild degree favors SOAP. Careful follow-up and late results are crucial. Because this is a severe but rare complication, a long-term database analysis is essential.

Protein-losing enteropathy (PLE) is a commonly experienced complication in the field of congenital heart disease. However, there are few reports on the PLE of non-Fontan cases compared with Fontan cases. We report two patients who developed PLE after an atrial switch operation for congenitally corrected transposition of the great arteries. Each of them received surgical reconstruction of the obstructed inferior caval pathway and balloon dilatation of the superior caval pathway, respectively, and remissions of PLE were obtained for both cases. Based on previous literature, we reviewed 12 PLE cases after atrial switch operation, including our cases, and 11 (92%) of them had stenosis or complete occlusion of the intra-atrial baffles. The sites of stenosis were superior vena cava in 5 cases, inferior vena cava in 2 cases, and both in 4 cases. All 11 patients with baffle obstruction were treated with surgical or transcatheter treatments and enhancement in PLE was observed in 8 of 11 patients (73%). Baffle obstruction is a major cause of PLE after atrial switch operation, and either superior vena cava or inferior vena cava obstruction can trigger PLE. Surgical or transcatheter intervention for stenotic regions is highly efficient and should be the first-line treatment option.

We present a male infant diagnosed with pulmonary atresia, hypoplastic right ventricle, ventricular septal defect, atresia of the orifice of the left coronary artery, and right ventricle-dependent coronary circulation. We assessed the blood flow in the sinusoidal communication (SC) utilizing pulsed-wave Doppler echocardiography to calculate coronary perfusion during the clinical course. At the time of delivery, the SC blood flow had a biphasic pattern; antegrade from the right ventricle (RV) to the left ventricle (LV) intramyocardium during systole and retrograde from the LV intramyocardium to the RV during diastole. The patient received a Blalock–Taussig (BT) shunt method at the age of 1 month. Following the BTS method, myocardial ischemia developed, and the SC blood flow showed a triphasic pattern; antegrade from the RV to the LV intramyocardium during systole, retrograde from the LV intramyocardium to the RV during early diastole, and antegrade from the RV to the LV intramyocardium during late diastole. The increase in diastolic RV pressure after the BTS method caused a reversal of the late diastolic blood pressure between the RV and LV myocardium, which was considered to induce a flow of non-oxygenated flow through the SC, leading to LV myocardial ischemia. An aorta-to-right ventricle (Ao-RV) shunt method was conducted at the age of 2 months. The SC blood flow revealed the same triphasic pattern, but the myocardial ischemia vanished. We hypothesize that the increased oxygenation of the SC blood flow during late diastole from the Ao-RV shunt enhanced the coronary circulation. Evaluation of SC blood flow patterns is effective for predicting myocardial ischemia.

Treatment of patent ductus arteriosus is crucial for the survival of patients with ductus arteriosus (DA)-dependent congenital heart diseases. Prostaglandin E₁ (PGE₁) agents are commonly used to keep the DA open; however, maintaining an appropriately sized DA is difficult. We saw a patient with hypoplastic left heart syndrome (HLHS) and critical pulmonary stenosis (cPS) whose DAs had become narrow and were re-dilated by milrinone. The patient with HLHS had bilateral pulmonary artery banding and was awaiting the Norwood procedure. The diameter of the DA instantly decreased from 6.4 mm to 3.0 mm at 18 days of age during the administration of lipo-PGE₁. Although the administration of CD-PGE₁ was begun instead of lipo-PGE₁, the DA remained narrow. Therefore, we initiated the administration of milrinone at a dosage of up to 0.2 µg/kg/min. The DA quickly re-dilated to a sufficient size and remained open until the Norwood procedure. The patient with cPS was scheduled for percutaneous transluminal pulmonary valvuloplasty (PTPV). The diameter of the DA decreased from 3.7 mm to 1.2 mm at 3 days of age. The agent used was changed from lipo-PGE₁ to CD-PGE₁, but the DA remained narrow. As a result, we began administering milrinone and gradually increased the dosage to 0.4 µg/kg/min. The DA dilated after the initiation of milrinone treatment and remained open until the scheduled PTPV date. A narrow DA is a critical problem for patients with DA-dependent congenital heart disease, and additional treatments are required to avoid DA closure. Milrinone is another option besides PGE₁ agents to re-dilate the DA.

A 12 years-old boy was identified with coarctation of the aorta and ventricular septal defect at birth. He received extended aortic arch anastomosis for the coarctation of the aorta at the age of 1 month. He required a treatment with balloon angioplasty postoperatively for recurrent coarctation twice. On the other hand, recurrent coarctation subsequently occurred and severe aortic valve regurgitation also emerged. The recurrent coarctation developed to a minimum diameter of 5 mm and a pressure gradient of 50 mmHg across a total length of 4 cm of stenotic lesion by close examination at the age of 11 years. Moreover, severe aortic valve regurgitation was found. He had descending thoracic aortic replacement, aortic valve replacement, and ventricular septal defect closure through combined median sternotomy and left antero-lateral thoracotomy, taking considering factors such as anticipated severe adhesion around the aorta, secured establishment of selective cerebral perfusion and facilitated intracardiac repair. The postoperatively, the pressure gradient between the upper and lower limbs was diminished. Although catheter intervention and extraanatomical bypass have been variably reported for patients with recurrent coarctation, these procedures leave specific concerns in the late term. On the other hand, combined median sternotomy and left antero-lateral thoracotomy could facilitate the intracardiac repair and allow us to complete a highly curative procedure conforming anatomical repair of coarctation at the expense of seemingly excessive invasiveness.

Heparin doses are typically maintained at 180–200 s as activated clotting time (ACT) to assess anticoagulant therapy for venous arterial extracorporeal membrane oxygenation (V-A ECMO) in children. However, it is challenging to control the proper heparin dose during V-A ECMO with ACT clinically, which could result in fatal hemorrhagic complications such as cerebral hemorrhage. Although Viscoelastic hemostatic assay (VHA) device: TEG^® 6s (Haemonetics Corp., Boston, USA) could be employed to determine the proper heparin dose during adult ECMO, there is no report about whether TEG^® 6s could be effective for control of heparin therapy during ECMO in children. In our report, we discussed the clinical use of TEG^® 6s for control of heparin therapy during ECMO in children. A 6-month-old female infant with a weight of 6.6 kg was scheduled to undergo an operation for supravalvular and coronary artery stenosis. Because heart failure worsened rapidly, V-A ECMO was needed for circulatory support. We observed that ACT and TEG^® 6s revealed conflicting findings of heparin dose during ECMO. On the second hospital day, ACT was 130–150 s, which requested an increase in heparin. However, TEG^® 6s demonstrated that the CK R-CKH R value was above the upper limit of measurement, which showed excessive heparin dose. We decided that heparin dose was reduced on the basis of TEG^® 6s values. Eventually, V-A ECMO could have been used for 10 days without thrombotic, including intra-circuit thrombus and bleeding complications. Our report revealed the possibility that TEG^® 6s might be better device to decide the proper heparin dose during ECMO in children than the ACT.