Cardiopulmonary exercise testing (CPET) is a clinical testing that allows for the analysis of gas exchange during exercise. Maximal oxygen uptake (V̇O₂max), a comprehensive index of cardiac, respiratory, and skeletal muscular functions, is one of the important measurements obtained in CPET. Anaerobic threshold, the exercise intensity at which lactate starts to accumulate in the blood, is widely used, as it represents an exercise level that is not too strenuous, but not too mild. The minute ventilation/carbon dioxide output relationship (V̇E/V̇CO₂ slope) is often used as a prognostic marker of cardiac failure. The oxygen uptake efficiency slope (OUES) is a submaximal index of cardiorespiratory functional reserve, and correlates strongly with V̇O₂max. This marker is utilized as a prognostic tool for various heart diseases including congenital heart defects.

Background: Total anomalous pulmonary venous connection (TAPVC) has several morbid conditions. Particularly, treatment outcomes of patients with TAPVC who postoperatively develop pulmonary venous obstruction (PVO) remain unsatisfactory. We investigated the usefulness of 320-row multidetector computed tomography (CT) imaging for early detection of postoperative PVO.

Methods: We included 18 consecutive patients who postoperatively underwent chest CT using 320-row multidetector CT during June 2007–December 2018 in Iwate Medical University Hospital.

Results: TAPVC types were supracardiac (in 3 patients), cardiac (4), infracardiac (6), and mixed (5). PVO was found preoperatively in 10 patients and postoperatively in 4. Median age and radiation exposure dose were 133 (33–556) days and 0.65 (0.40–1.32) mSv, respectively, when postoperative CT was employed. The anastomotic site between the common chamber and the atrium was located in front of the spine in 9 patients (central type), whereas it was deviated to the right/left side of the spine with the pulmonary vein (PV) coursing over the descending aorta and the spine in the remaining 9 patients (lateral type). Among those, CT revealed PV flattening and anastomotic stenosis in 9, and 4 of them were subjected to surgical PVO relief due to significant pulmonary congestion. In two of the four patients reoperated, PVO clinically developed eventually; PVO had not been recognized initially due to limited imaging window and presumably compromised PV flow at the affected site.

Conclusions: 320-row multidetector CT with a low radiation exposure dose could aid in early diagnosis of PVO in the peripheral region.

Background: Establishment of a dual coronary arterial system for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) results in good early and late outcomes. Left ventricular (LV) function often improves to normal late after ALCAPA repair. The impact of age at operation, nonetheless, remains unknown on the long-term outcomes and recovery process of LV function and dimension.

Methods: This retrospective review included 11 patients with ALCAPA who underwent surgery from 2003 to 2018. Patients were divided into two groups based on age at ALCAPA repair: group Y (n=5, age ≤1 year) and group O (n=6, age >1 year). Early, mid-term, and long-term outcomes and recovery of LV function and dimension were assessed. Median age at repair for groups Y and O was 4.3 and 94 months, respectively.

Results: No in-hospital or after-discharge deaths occurred in either of the groups. Median follow-up was 13.4 (1.8–16.4) years. Ejection fraction and fractional shortening in group Y took a longer interval (a median 5.8 months) before catching up with the level in group O, albeit with no statistical significance. No significant differences were found between the two groups in LV recovery after the mid-term follow-up.

Conclusions: We achieved excellent early and late outcomes with a low reintervention rate, regardless of age at ALCAPA repair. The LV function and dimension in group Y tended to need a few months longer to improve compared with those in group O.

Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) is widely used for severe circulatory failure and can be deployed quickly and easily even in small infants. Cardiac function, however, does not recover soon in some patients, who eventually die when the heart becomes unable to pump the blood out through the aortic valve, end-diastolic pressure of the left ventricle (LV) is elevated, pulmonary venous congestion progresses and myocardial recovery is disturbed in the LV. In this situation, LV decompression is of practical use. We report herein a successful case of aortic valvuloplasty in a female infant requiring ECMO with LV venting for severely reduced LV function and LV hypertrophy. LV distention and pulmonary congestion were prevented, and the patient was weaned off ECMO. Decompressing the left side of the heart in a timely manner may be essential according to the patient’s hemodynamic profile.

Two neonates were treated for a combination of a distal type of anomalous origin of the right pulmonary artery from the ascending aorta and aortic coarctation. Aortic arch reconstruction was performed using an extended aortic arch anastomosis in both cases. In the first case, the right pulmonary artery was reconstructed by a direct anastomosis behind the ascending aorta. Early reoperation for graft interposition was required to treat stenosis at the proximal right pulmonary artery due to a residual ductal tissue there. In the second case, such ductal tissues were sufficiently resected at the initial repair, and the right pulmonary artery was reconstructed using an artificial graft anterior to the ascending aorta. As aortic arch reconstruction for aortic coarctation reduces the space posterior to the ascending aorta, an appropriate route should be considered sensibly for the right pulmonary artery to be reconstructed.

We report a case of a 4-year-old boy with preceding Kawasaki disease (KD) who presented with acute myocardial infarction (AMI) caused by giant coronary artery aneurysms, a serious complication of KD. Echocardiography at the time of AMI presentation revealed dyskinesis of the apex. He had been on aspirin (85 mg), dipyridamole (50 mg) and warfarin (6.5 mg) orally since the previous KD event. He was transferred to our facility more than 18 hours after the onset of the AMI symptom. Given this delay in transfer and his international normalized ratio of 2.55 on admission, we did not consider percutaneous coronary intervention or thrombolytic therapy was appropriate. Therefore, we simply gave IV heparin infusion. His symptoms improved from that day forward and he was discharged on day 68. At follow-up, ventricular contraction around the apex gradually improved, and enhanced computed tomography showed recanalization of the once occluded vessel. Six months after the AMI diagnosis, the patient underwent coronary artery bypass grafting for better coronary circulation. The postoperative course was uneventful, and cardiac function improved still further.

Cardiovascular beriberi may occur because of an unbalanced diet or eating disorder, and can lead to circulatory insufficiency. Here, we report a 3-year-old-boy with avoidant/restrictive food intake disorder who developed severe pulmonary artery hypertension (PAH) due to cardiovascular beriberi and was treated with a phosphodiesterase type 5 inhibitor in addition to thiamine. The patient had limited oral intake for one month due to a mouth ulcer. On admission, he was sluggishness, hypotensive, tachycardic and tachypneic. Severe PAH was suspected based on echocardiography showing enlargement of the right ventricle and the right atrium and deformity of the interventricular septum. A sildenafil administration test was performed under oxygen administration by high flow nasal cannula; PAH improved one hour later. Based on the dietary history, thiamine was started for possible cardiovascular beriberi due to vitamin B1 deficiency which was eventually confirmed by the blood sample on admission. Since idiopathic PAH could not be ruled out initially, tadalafil was started on the day of admission. With these treatments, oral intake increased. Tadalafil was gradually reduced and eventually discontinued 7 months later. Cardiac catheterization 8 months after the onset of the PAH episode did not show severe PAH. This case indicates that a potential of PAH due to cardiovascular beriberi should be kept in mind if the physical condition deteriorates in a child with an eating disorder or unbalanced diet. Important issues are timely assessment using echocardiography, treatments according to appropriate understanding of pathophysiology, and supplementation of vitamin B1.