Kiyohiro Takigiku

Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of patients with hypoplastic left heart syndrome with and without prenatal diagnosis

Abstract Background: As prenatal diagnosis of congenital heart disease has gained in popularity, the questions of whether prenatal diagnosis of congenital heart disease is beneficial for the patient and whether fetal echocardiography has improved the prognosis of congenital heart disease are arising.

Normal values and clinical relevance of left atrial myocardial function analysed by speckle-tracking echocardiography: multicentre study

AIMS The aim of this multicentre study was to determine the normal range and the clinical relevance of the myocardial function of the left atrium (LA) analysed by 2D speckle-tracking echocardiography (2DSTE). METHODS AND RESULTS We analysed 329 healthy adult subjects prospectively included in 10 centres and a validation group of 377 patients with left ventricular diastolic dysfunction (LVDD). LA myocardial function was analysed by LA strain rate peak during LA contraction (LA-SRa) and LA strain peak during LA relaxation (LA-Strain). The range of values of LA myocardial function in healthy subjects was LA-SRa -2.11 ± 0.61 s(-1) and LA-Strain 45.5 ± 11.4%, and the lowest expected values of these LA analyses (calculated as -1.96 SD from the mean of healthy subjects) were LA-SRa -0.91 s(-1) and LA-Strain 23.1%. Concerning the clinical relevance of these LA myocardial analyses, LA-SRa and LA-Strain detected subtle LA dysfunction in patients with LVDD, even though LA volumetric measurements were normal. In addition, in these patients we found that the functional class (dyspnoea-NYHA classification) was inversely related to both LA-Strain and LA-SRa. CONCLUSION In the present multicentre study analysing a large cohort of healthy subjects and patients with LVDD, the normal range and the clinical relevance of the myocardial function of the LA using 2DSTE have been determined.

Multidirectional Global Left Ventricular Systolic Function in Normal Subjects and Patients with Hypertension: Multicenter Evaluation

BACKGROUND The aim of this multicenter study was to determine the normal ranges and the clinical relevance of multidirectional systolic parameters to evaluate global left ventricular (LV) systolic function. METHODS Three hundred twenty-three healthy adult subjects prospectively included at 10 centers and a cohort of 310 patients with hypertension were analyzed. Multidirectional global LV systolic function was analyzed using two-dimensional speckle-tracking echocardiography by means of two indices: longitudinal-circumferential systolic index (the average of longitudinal and circumferential global systolic strain) and global systolic index (the average of longitudinal, circumferential, and radial global systolic strain). RESULTS The ranges of values of the multidirectional systolic parameters in healthy subjects were -21.22 ± 2.22% for longitudinal-circumferential systolic index and 29.71 ± 5.28% for global systolic index. In addition, the lowest expected values of these multidirectional indices were determined in this population (calculated as -1.96 SDs from the mean): -16.86% for longitudinal-circumferential systolic index and 19.36% for global systolic index. Concerning the clinical relevance of these measurements, these indices indicated the presence of subtle LV global systolic dysfunction in patients with hypertension, even though LV global longitudinal systolic strain and LV ejection fraction were normal. Moreover, in these patients, functional class (dyspnea [New York Heart Association classification]) was inversely related to both the longitudinal-circumferential index and the global systolic index. CONCLUSIONS In the present multicenter study analyzing a large cohort of healthy subjects and patients with hypertension, the normal range and the clinical relevance of multidirectional systolic parameters to evaluate global LV systolic function have been determined.

Do we need fenestration when performing two-staged total cavopulmonary connection using an extracardiac conduit?

Between August 1999 and December 2007, 72 consecutive patients with single ventricle physiology underwent a modified Fontan procedure after a bidirectional Glenn shunt using an extracardiac polytetrafluoroethylene conduit without fenestration. Nitric oxide gas inhalation was commenced just after cardiopulmonary bypass together with intravenous phosphodiesterase III inhibitor administration. After oral intake was started, pulmonary vascular dilators such as beraprost, sildenafil, bosentan were given orally according to amount of chest drainage and patients condition. After discharge, oxygen therapy at home was continued for three months. No hospital death occurred after surgery. All patients were followed by our institute and follow-up period was 44.2+/-26 (36-106.8) months. One late death occurred during this follow-up period after re-operation. Cardiac catheterization after the Fontan completion showed transpulmonary gradient of 5.9+/-2.4 mmHg, systemic output of 3.4+/-2.1 l/min m2. Arterial oxygen saturation (SaO(2)) at the latest outpatient visit was 94.4+/-3.8%. According to our clinical experience with two-staged total cavopulmonary connection using an extracardiac conduit without fenestration, fenestration in the Fontan circuit is not necessary when performing the Fontan completion. Two-staged extracardiac total cavopulmonary connection without fenestration can be satisfactorily completed with the aid of pulmonary vasodilation therapy.

Sildenafil reduces pulmonary vascular resistance in single ventricular physiology

BACKGROUND High pulmonary vascular resistance (PVR) may be a risk factor for early and late mortality in both Glen shunt and Fontan operation patients. Furthermore, PVR may increase long after the Fontan operation. Whether pulmonary vasodilators such as phosphodiesterase 5 inhibitors can decrease PVR in patients with single ventricular physiology remains undetermined. METHODS AND RESULTS This was a prospective, multicenter study. Patients with single ventricular physiology who have a PVR index higher than 2.5 Wood units·㎡ (WU) were enrolled. Cardiac catheterization was performed before and after administration of sildenafil in all patients. After the Fontan operation, a six minute walk test (6MWT) was also performed. A total of 42 patients were enrolled. PVR was significantly decreased in each stage of single ventricular physiology after sildenafil administration: from 4.3±1.5WU to 2.1±0.6WU (p<0.01) in patients before a Glenn shunt, from 3.2±0.5WU to 1.6±0.6WU (p<0.001) in patients after a Glenn shunt, and from 3.9±1.7WU to 2.3±0.8WU (p<0.001) in patients after Fontan. In patients after Fontan, the 6MWT increased from 416±74m to 485±72m (p<0.01), and NYHA functional class improved significantly (p<0.05) after sildenafil administration. No major side effects were observed in any patients. CONCLUSIONS Sildenafil reduced PVR in patients with single ventricle physiology. Sildenafil increased exercise capacity and improved NYHA functional class in patients after a Fontan operation. This implies that pulmonary vasodilation is a potential therapeutic target in selected patients with elevated PVR with single ventricle physiology. Long-term clinical significance warrants further study.

Second-Stage Palliation After Bilateral Pulmonary Artery Bands for HLHS and its Variants—Which is Better, Modified Norwood or Norwood Plus Bidirectional Glenn?

Background. The purpose of this study was to evaluate the surgical outcomes and pulmonary artery (PA) development associated with a new strategy wherein the modified Norwood (N) procedure is performed at 1-2 months after bilateral pulmonary artery banding (PAB). Methods. Between January 2008 and February 2010, 16 patients underwent Norwood-type operation after previous bilateral PAB. For analysis, patients were divided into two groups. Group I (n = 11) underwent modified Norwood procedure with either right modified Blalock Taussig (RMBT) shunt (n = 4) or right ventricle to pulmonary artery (RV-PA) conduit (n = 7). Group II (n = 5) underwent Norwood procedure plus bidirectional Glenn anastomosis. Diagnoses were hypoplastic left heart syndrome in 6 and its variants in 10. Results. There was no surgical death and no late death. Pulmonary artery interventions were performed at the time of the Norwood procedure in 27% in Group I and in 100% in Group II (p < 0.05). Additional PA interventions were performed during the period of follow-up in 4 cases in Group I (36.4%), and in 4 cases in Group II (80.0%). Additional Blalock Taussig shunts were performed in 7 patients, resulting in significant increase in PA index. In all, four patients have reached total cavopulmonary connection, and one has undergone biventricular repair. Eight patients in Group I and one patient in II Group reached bidirectional Glenn anastomosis. In Gp II, two patients showed LPA narrowing or obstruction with PA index of 80 ± 12 mm2/m2. Conclusions. Regarding the second-stage palliation after bilateral PAB, modified Norwood procedure with either RMBT or RV-PA conduit has some advantages compared with Norwood plus BDG with respect to subsequent pulmonary artery development. Additional BT shunt may contribute to PA development, even in the patients with Norwood procedure with RV-PA conduit.

Successful transluminal angioplasty of renal arterial stenosis using the transcarotid approach

We successfully performed percutaneous transluminal angioplasty to treat severe renovascular hypertension with left ventricular failure in a 5-month-old infant. Using the transcarotid approach, we dilated the stenotic left renal artery without any difficulties, using progressively larger balloons designed for dilation of coronary arteries.

Interventional treatment for fetus and newborn infant with congenital heart disease.

Abstract Background : Catheter intervention has become popular not only in adult patients but in younger patients with congenital heart disease. The early neonatal catheter interventional treatment has also been attempted in conjunction with the development of prenatal diagnosis of the congenital heart disease.

Unilateral absence of right proximal pulmonary artery in the setting of severe pulmonary hypertension: considerations for surgical repair and perioperative management

We report a successfully treated case of unilateral absence of a pulmonary artery, associated with an atrial septal defect and chronic lung disease with severe pulmonary hypertension. Because this severe pulmonary hypertension could jeopardize postoperative hemodynamic, the ingenuity of surgical strategy was required. The atrial septal defect was left open as a safety “pop-off” valve, and prosthetic graft was chosen as a reconstructive material to avoid excessive dilatation from exposure to unpredictable postoperative pulmonary hypertension.

Measurement of Aortic Valve Coaptation and Effective Height Using Echocardiography in Patients with Ventricular Septal Defects and Aortic Valve Prolapse

Decreased coaptation height in adults has been identified as a marker of early valve failure. We evaluated aortic valve coaptation and effective height in healthy children and in children with a ventricular septal defect (VSD) with aortic cusp prolapse (ACP), using echocardiography. We included 45 subjects with VSD with ACP, 27 did not develop aortic regurgitation (AR) by ACP and 18 developed AR by ACP, and 83 healthy children as controls. Aortic root anatomy was estimated using the parasternal long-axis view. We measured the diameter of aortic valve (AV), coaptation height (CH), and effective height (EH) of the aortic valve. We defined the ACH (CH/AV ratio) and AEH (EH/AV ratio) indices as follows: