Long-Term Renal Involvement in Association with Fontan Circulation.

Multiorgan dysfunction is a concern of Fontan patients. To clarify the pathophysiology of Fontan nephropathy, we characterize renal disease in the long-term observational study. Medical records of 128 consecutive Fontan patients [median age: 22 (range 15-37) years old] treated between 2009 and 2018 were reviewed to investigate the incidence of nephropathy and its association with other clinical variables. Thirty-seven patients (29%) showed proteinuria (n = 34) or < 90 mL/min/1.73 m2 of estimated glomerular filtration rate (eGFR) (n = 7), including 4 overlapping cases. Ninety-six patients (75%) had liver dysfunction (Forns index > 4.21). Patients with proteinuria received the Fontan procedure at an older age [78 (26-194) vs. 56 (8-292) months old, p = 0.02] and had a higher cardiac index [3.11 (1.49-6.35) vs. 2.71 (1.40-4.95) L/min/m2, p = 0.02], central venous pressure [12 (7-19) vs. 9 (5-19) mmHg, p < 0.001], and proportion with > 4.21 of Forns index (88% vs. 70%, p = 0.04) than those without proteinuria. The mean renal perfusion pressure was lower in patients with a reduced eGFR than those without it [55 (44-65) vs. 65 (45-102) mmHg, p = 0.03], but no other variables differed significantly. A multivariable analysis revealed that proteinuria was associated with an increased cardiac index (unit odds ratio 2.02, 95% confidence interval 1.12-3.65, p = 0.02). Seven patients with severe proteinuria had a lower oxygen saturation than those with no or mild proteinuria (p = 0.01, 0.03). Proteinuria or a decreased eGFR differentially occurred in approximately 30% of Fontan patients. Suboptimal Fontan circulation may contribute to the development of proteinuria and reduced eGFR.

Usefulness of a Radiofrequency Transseptal Needle in the Second Puncture of Fontan Extracardiac Conduit.

We performed a second puncture of the extracardiac conduit in an 11-year-old Fontan patient to assess the patency of the stent previously deployed in the left pulmonary vein. For the first puncture, a mechanical Brockenbrough needle was selected to puncture the Gore-Tex conduit, an electrical insulator. For the second puncture, the location of that previous hole was detected as an indentation covered with atrial tissue, which is an electrical conductor. The second puncture was performed safely using a radiofrequency transseptal needle.

A case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricular fistula.

Tricuspid atresia with pulmonary valve absence is a rare malformation characterized by left ventricular outflow obstruction due to asymmetrical ventricular septal hypertrophy and associated with a poor prognosis. Coexisting coronary-right ventricular fistula is rarely described. We encountered a case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricle fistula that successfully underwent Fontan completion. Right ventricle dilatation due to coronary-right ventricle fistula was observed in addition to mass like ventricular septal hypertrophy protruding into the left ventricular outflow tract. Right ventricle reduction and prevention of progressive left ventricular outflow obstruction were achieved by closure of the coronary-right ventricle fistula closure and plication of the right ventricle with the bidirectional Glenn procedure.

Percutaneous major aortopulmonary collateral artery banding using a covered stent in an infant.

We report a 6-month-old infant with pulmonary atresia with ventricular septal defect who was successfully treated with a novel technique of percutaneous major aortopulmonary collateral artery (MAPCA) banding with a covered stent. He suffered from heart failure due to a residual MAPCA of 4.5 mm in diameter. A covered stent tied with 2 5-0 size nylon threads was successfully deployed into the MAPCA in a dumbbell shape with a banding diameter of 2.8 mm. Banding covered stent implantation is a useful method to reduce the pulmonary blood flow in patients with MAPCA, which could be a less-invasive alternative to surgery.

Effective infliximab therapy for the early regression of coronary artery aneurysm in Kawasaki disease.

BACKGROUND: There is limited information available regarding the role of infliximab (IFX) following the acute phase of Kawasaki disease (KD). We aimed to evaluate whether IFX is associated with coronary artery aneurysm (CAA) regression. METHODS: Between 2005 and 2016, we identified 971 consecutive patients with KD from 3 tertiary institutions, and 49 (5%) with CAAs were enrolled in our study. Patients were divided into 2 groups: 27 who received IFX and 22 who did not. The persistence rate of CAAs was compared between the groups. RESULTS: Age, sex, and duration of the febrile period did not significantly differ between the groups. The maximum value of C-reactive protein was higher in the IFX- than in the non-IFX group. The maximum z-score of CAAs did not differ between the groups. The 2-, 4- and 6-year cumulative persistence rate of CAA was 24%, 24% and 24% in IFX-group, whereas 67%, 52% and 33% in non-IFX group, respectively (P = 0.03). The median duration of CAA regression was 1.1 vs. 4.6 years. Among those who developed medium- or large-sized CAAs, the 2-, 4- and 6-year cumulative persistence rate of CAA was 33%, 33% and 33% in IFX group, whereas 77%, 51% and 48% in non-IFX group, respectively (P = 0.047). Multivariate logistic regression analysis indicated that the maximum z-score (hazard ratio 0.72, p < 0.001) and response to IFX (hazard ratio 4.56, p = 0.017) were independently related to regression. CONCLUSION: IFX therapy was observed to be effective for the early improvement of CAAs in patients with intravenous immunoglobulin-resistant KD.

Takotsubo cardiomyopathy associated with Paragonimiasis westermani.

An 11-year-old boy collapsed during morning assembly at his junior high school. The automated external defibrillator detected ventricular fibrillation and provided shock delivery. He was successfully resuscitated and reverted to sinus rhythm. Electrocardiography showed ST-T elevation in the precordial leads. Echocardiography and angiography demonstrated akinesia of the apex and mid-wall of the left ventricle with preserved contraction of the basal segments, which suggested Takotsubo cardiomyopathy. The patient and his family had often eaten uncooked crab, and his father had a past history of infection with Paragonimiasis westermani. The patient had had a persistent cough and chest pain for several weeks. Chest radiograph showed cystic cavities in the left upper lung. Microbiological examination of the sputum demonstrated an egg of P. westermani and immunological assay showed a raised antibody titre to P. westermani. On the12th day of admission, he developed seizures, and magnetic resonance imaging demonstrated cerebral involvement. After the administration of praziquantel for 3 days, the clinical manifestations improved immediately, and echocardiography normalised within 3 weeks. The patient was discharged on the 32nd day + and follow-up was normal. Takotsubo cardiomyopathy following a potentially fatal arrhythmia is a rare cardiac complication associated with pulmonary and central nervous system infection by P. westermani.

Intravascular Ultrasound-Guided Cardiac Intervention in a Fontan Patient with Allergy to Contrast Media.

Allergy to contrast media is hazardous in patients who need multiple cardiac catheterizations. We describe the case of a 16-year-old boy who presented with fatigue and cyanosis. He had undergone a Fontan operation 14 years previously, after which he developed an allergy to iodinated contrast media. Contrast echocardiograms by manual injection of microbubbles suggested the presence of a venovenous collateral vessel from the brachiocephalic vein draining into the pulmonary vein. We used intravascular ultrasound as the sole imaging method to identify the target vessel and to guide deployment of an Amplatzer Vascular Plug II. The patient experienced immediate improvement in his systemic saturation. Our experience shows that intravascular ultrasound may be a feasible alternative to standard imaging methods in patients with congenital heart disease who need cardiac interventional procedures and are allergic to contrast media.

Bilateral Coronary-Pulmonary Artery Fistulas in Pulmonary Atresia With Ventricular Septal Defect.

We present a very rare case of bilateral coronary to pulmonary artery fistulas associated with pulmonary atresia with ventricular septal defect. The courses of coronary to pulmonary artery fistulas have to be clearly delineated by detailed angiography prior to corrective surgery.

Resection of Kommerell Diverticulum After the Arterial Switch for TGA With Bilateral PDAs and Right Aortic Arch.

We present a very rare case of bilateral ductus arteriosus in transposition of the great arteries with right aortic arch and aberrant retroesophageal left subclavian artery (SCA). Around 1 month after the successful arterial switch operation, the baby showed wheezing and retractive breathing. The computed tomography revealed that trachea and esophagus were sandwiched between the posterior displaced ascending aorta and the origin of the retroesophageal aberrant left SCA, the so-called Kommerell diverticulum (KD). This compression was successfully relieved by resection of the KD and division of the retroesophageal aberrant SCA through right thoracotomy.

Relationship between pulmonary arterial resistance and compliance among patients with pulmonary arterial hypertension and congenital heart disease.

BACKGROUND: It is unknown whether changes in pulmonary arterial resistance (Rp), pulmonary arterial compliance (Cp), and the product of Rp and Cp (the RC time) in patients with pulmonary arterial hypertension (PAH) are related to an increase in pulmonary blood flow. The aim of this study is to clarify relationships between these parameters before and after corrective surgery among patients with PAH and congenital heart disease. METHODS: We performed cardiac catheter examinations and compared Rp, Cp, and the RC time before and after corrective surgery in 100 infants (53 boys) with PAH related to ventricular septal defect. RESULTS: Median age at surgery was 2.9 (0.6-28.5) months. Preoperative values of the ratio of pulmonary to systemic blood flow (Qp/Qs), systolic pulmonary arterial pressure (PAP), Rp, Cp, and the RC time were 3.5 (2.7-4.3), 65 (56-70) mm Hg, 2.18 (1.64-3.19) Wood unit/m(2), 2.67 (2.01-3.38) mL/mm Hg/m(2), and 0.36 (0.31-0.40) seconds, respectively. Postoperative systolic PAP, Cp, and the RC time were significantly decreased to 26 (23-29) mm Hg, 1.96 (1.77-2.26) mL/mm Hg/m(2), and 0.31 (0.26-0.36) seconds, respectively, although Rp remained unchanged at 2.53 (2.06-3.31) Wood unit/m(2). The relationship between Rp and Cp was inversely related before and after surgery, and the Rp-Cp coupling curve was shifted downward after surgery. In addition, lower preoperative Cp (partial regression coefficient = -3.35; P = .001) and preterm delivery (partial regression coefficient = 6.28; P = .02) were independently related to postoperative higher systolic PAP. CONCLUSIONS: Rp-Cp coupling depends on both the amount of pulmonary blood flow and the condition of the pulmonary vasculature. Lower preoperative Cp is an independent predictive factor to predict higher postoperative pulmonary systolic pressure leading to an increase in right ventricular workload. It is necessary to assess Rp-Cp coupling before surgery among patients with PAH related to congenital heart disease.