Catheter Intervention for Flow Regulatory Clips on Palliative Shunts and Conduits in Patients with Congenital Heart Disease.

Catheter intervention (CI) for a Blalock-Taussig shunt (BTS) or a ventricle-to-pulmonary artery conduit (VPC) is often required after a palliative surgery for congenital heart disease. Flow regulatory clips help improve interstage mortality; their use necessitates CIs to prevent cyanosis. To study the CI outcomes in patients who underwent palliative surgery with either BTSs or VPCs with flow regulatory clips. This single-center retrospective study evaluated demographic characteristics and interventional outcomes of 49 consecutive pediatric patients who required CI for BTS (BTS group) or VPC (VPC group) between January 2008 and September 2018. Overall, 34 and 18 procedures were performed in the BTS and VPC groups, respectively. Moreover, 19/32 (59.3%) and 12/17 (70.1%) patients from the BTS and VPC groups had flow regulatory clips, respectively. All clips were unclipped successfully; one patient in each group underwent staged unclipping. A higher proportion of "clipped patients" underwent CI due to desaturation [clipped vs. non-clipped: BTS, 10/20 (50.0%) vs. 3/14 (21.4%), p = 0.092; VPC, 9/13 (69.2%) vs. 1/5 (20.0%), p = 0.060]. Most clipped patients successfully progressed to the next stage [BTS, 19/20 (95.0%); VPC, 12/13 (92.3%)]. Severe adverse events (SAEs) were more frequent in the VPC group than in the BTS group [3/13 (23.1%) vs. 0/20 (0%), p = 0.024]. Two patients developed an atrioventricular block (requiring an atropine infusion), while one died due to pulmonary overcirculation. While the indication of CI was cyanosis for a higher proportion of clipped patients, all clips were unclipped successfully. The incidence of CI-related SAEs was higher in the VPC group than in the BTS group.

Pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries associated with hypoplastic left ventricle and severe mitral regurgitation.

We report the first successful Glenn procedure for pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries. This case was complicated by hypoplastic left ventricle and severe mitral regurgitation. Partial closure of the mitral valve at the time of Blalock-Taussig shunting resulted in improvement of heart failure.

Angiographic diagnosis for accurate assessment of congenital porto-systemic shunt and extrahepatic portal vein obstruction in children.

BACKGROUND: Congenital porto-systemic shunt (CPSS) is a rare disease and can cause fatal complications. Accurate angiographic assessment is mandatory for proper treatment. Although technically difficult, we developed assessment techniques and assessed their accuracy. One technique came from evaluating patients with extrahepatic portal vein obstruction (EHPVO). METHODS: We conducted a single center retrospective study to evaluate the efficacy of angiographic diagnostic procedure for the assessment of CPSS and EHPVO, and its impact on patients' subsequent interventions and clinical course. Eight patients with CPSS and two patients with EHPVO who underwent diagnostic angiography were included. Assessment of the intrahepatic portal vein was performed in all patients. The route of the shunt, and portal vein pressure under shunt occlusion, were also evaluated for patients with CPSS. Evaluation was first attempted with a balloon angiographic catheter (standard method). Three additional techniques were performed as needed: (i) direct wedge-catheter injection without balloon inflation, (ii) use of occlusion balloon in two patients, and (iii) hybrid angiography with sheath placement directly into the superior mesenteric vein. RESULTS: The standard method was sufficient in four patients. On the other hand, all three techniques were required in two patients each. One lost contact during follow up, but all other patients underwent optimal intervention. There were no complications related to the angiographic procedure. CONCLUSIONS: Use of direct wedge-catheter injection without balloon inflation, occlusion balloon, and hybrid catheterization improved the diagnostic yield in patients with CPSS or EHPVO.

Progression of left ventricular apical hypoplasia-like restrictive cardiomyopathy with severe pulmonary hypertension: Follow-up from fetal stage.

Left ventricular (LV) apical hypoplasia is a rare restrictive cardiomyopathy subtype with an unclear pathophysiology. LV apical hypoplasia typically presents with elongated right ventricle (RV) wrapping around a truncated and spherical LV with a deficient apex (the "banana-shape" of the RV). Here we report a case of a young boy with apparent LV apical hypoplasia that developed after birth; no "banana-shaped" RV was observed during the fetal period. Moreover, suprasystemic pulmonary hypertension (PH) developed even after a mitral valve replacement was performed for progressive mitral stenosis and regurgitation at 14 months of age. He underwent surgery for the Potts shunt, a shunt between the pulmonary artery and aorta, at 13 years to secure systemic output. His PH ameliorated and his heart failure remained stable for 3 years after the operation. This case indicates that the "banana-shaped" RV seen in this condition is not always congenital but that it can form and develop after birth. Furthermore, this case supports the usefulness of the Potts shunt as a therapeutic option in patients with severe PH due to LV apical hypoplasia. .

Successful transcatheter closure of coronary artery fistula in a patient with anomalous aortic origin of the left main coronary artery from the right aortic sinus.

Transcatheter closure is an established method to treat coronary artery fistula (CAF). We present transcatheter closure in a 6-year-old girl with CAF and anomalous aortic origin of the left main coronary artery from the right aortic sinus. The CAF originated from the left coronary artery (LCA), coursed through the interventricular septum (intraseptal course) with prominent dilation, and drained into the right ventricular outflow tract. She underwent transcatheter closure and was in a stable condition at the 3-year follow-up with regression of the dilated portion of the intraseptal-type LCA. Hence, transcatheter closure of CAF is feasible in patients with anomalous origins of coronary arteries. .

[Joint Cardiovascular Surgeries of Two Hospitals in Shizuoka for Adult Congenital Heart Disease (ACHD) Patients].

BACKGROUND: As for the medical management including surgery for the patients with adult congenital heart disease(ACHD), it may be difficult for most hospitals other than that have both adult/congenital cardiologists/cardiovascular surgeons. Between Shizuoka Prefectural General Hospital and Mt. Fuji Shizuoka Children's Hospital, medical stuff and information have been shared for these 4 years. And joint cardiovascular surgeries have started since 2015 autumn at Shizuoka Prefectural General Hospital. PURPOSE: The contents and the results of these joint operations were evaluated. PATIENTS AND METHODS: Thirteen joint operations were performed and median age at operation was 55 years old( male 3, female 10). The original diagnosis was tetralogy of Fallot/pulmonary atresia with ventricular septal defect 6, ventricular septal defect( VSD)±pulmonary stenosis 4, atrioventicular septal defect/two chamber right ventricle/Ebstein's anomaly 1 ( each). The procedures were pulmonary valve replacement/right ventricle out flow tract reconstruction 7, mitral valve plasty/tricuspid annuloplasty 4, Bentall 2, VSD closure 2 etc.(included multiple choices). RESULTS: There was no early mortality. One late mortality was occurred 17 months after the surgery due to acute myeloid leukemia. General conditions in other patients have been feasible and most of them were followed in Shizuoka Prefectural General Hospital. CONCLUSIONS: The results of the joint operations were feasible in the present study. Our joint project may become more important in the future.

Cardiac fetal ultrasonographic parameters for predicting outcomes of isolated left-sided congenital diaphragmatic hernia.

BACKGROUND: The purpose of this study was to investigate fetal cardiac ultrasonographic parameters in relation to the outcomes of patients with isolated left-sided congenital diaphragmatic hernia (CDH). METHODS: Subjects comprised 33 fetuses with fetal CDH and 99 control fetuses without cardiac and lung anomalies. Three parameters, cardiothoracic area ratio (CTAR), the ratio of the diameter of the main pulmonary artery to that of the ascending aorta (MPA/AAo), and the ratio of the diameter of the tricuspid valve to that of the mitral valve (TV/MV) were compared between fetal CDH and control subjects and between survival (SG) and non-survival (NSG) groups. RESULTS: All three parameters differed significantly between CDH and control fetuses. Significant differences between SG and NSG were observed in CTAR and TV/MV ratio, but not in MPA/AAo ratio before and after 32 gestational weeks. A significant increase in the TV/MV ratio was noted along with an increase in gestational age. A TV/MV ratio of >1.72 discriminated non-survivors from survivors with better sensitivity and specificity. CONCLUSIONS: Fetal cardiac ultrasonography provides useful information for predicting the outcomes of isolated left-sided CDH. The TV/MV ratio was considered a reliable indicator that reflected outcomes of isolated left-sided CDH.

Case studies of patients successfully and unsuccessfully managed pre- and post-Fontan procedure.

As a result of advances and modifications in surgical procedures and the development of drugs for pulmonary arterial hypertension, many patients who have undergone Fontan procedures are able to enjoy good quality of life, without pulmonary arterial hypertension and severe complications. In Shizuoka Children's Hospital, drugs for pulmonary arterial hypertension have long been given to Fontan candidates and patients with established Fontan circulation to maintain sufficient pulmonary blood flow and suppress pulmonary arterial hypertension. We present three typical cases that were treated with anti-pulmonary hypertensive drugs before or after Fontan procedure. The first case had asplenia syndrome, and a single ventricle with major aortopulmonary collateral arteries. Anti-pulmonary hypertensive therapy permitted a Fontan procedure and maintained a good long-term quality of life. The second case was a Down syndrome patient who had progressive cyanosis after a Fontan operation. Anti-pulmonary hypertensive therapy improved cyanosis. The third case suffered from protein-losing enteropathy, for which all procedures and medical therapies were ineffective. Fontan candidates and patients with Fontan circulation have varied anatomical backgrounds and pulmonary properties. We must identify the conditions that lead to successful Fontan procedure and Fontan circulation correction, as well as conditions that result in failed Fontan procedure and poorly-controlled Fontan circulation.

Prenatally diagnosed hypoplastic left heart syndrome with intact atrial septum and ventriculocoronary arterial fistula.

Intact atrial septum (IAS) and left ventriculocoronary arterial fistula (LVCAF) are the significant risk factors of hypoplastic left heart syndrome (HLHS), and when they are present, prognosis is grim. We report a case of prenatally diagnosed HLHS with IAS and LVCAF that was treated with surgical atrial-septostomy and bilateral pulmonary arterial banding (BPAB) immediately after birth. The Doppler flow pattern of left anterior descending coronary artery (LAD) with dominant retrograde flow was normalized after atrial-septostomy with decompression of left atrial (LA) hypertension. This is the first report showing the influence of atrial-septostomy on coronary flow in HLHS patient with LVCAF.