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Introduction: It is reported that pressure wave reflection is enhanced by external compression of the femoral artery. Therefore, it is possible that cardiac catheterization itself can influence the aortic pressure waveform., Aim: The purpose of this study is to clarify the influence of sheath placement in a femoral artery on the pressure waveform., Methods: This study enrolled 21 pediatric patients (5.1±4.0years) who underwent cardiac catheterization. A sheath was placed in the femoral arteries of all patients. The change in the pressure waveform induced by the placement of the sheath was investigated using the b/a and d/a ratio of second derivative of a fingertip photoplethysmogram. A high b/a ratio means a stiff aorta and a low d/a ratio represents an enhancement of the aortic pressure wave reflection., Results: By the placement of the sheath in their femoral arteries, the b/a ratio was not influenced (sheath (-): -0.556±0.081 vs. sheath (+): -0.558±0.072; p=0.896). However, the d/a ratio was significantly decreased (-0.150±0.074 vs. -0.185±0.084; p=0.0003)., Conclusions: The placement of the femoral arterial sheath enhances the pressure wave reflection and would lead to a change in the central aortic pressure waveform., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: Recent studies showed that children with univentricular heart have elevated plasma B-type natriuretic peptide (BNP) levels prior to bidirectional cavopulmonary anastomosis (BDCPA). However, it remains to be established whether BNP levels reflect the degree of hemodynamic overload and relate to long-term outcome in univentricular circulation., Methods: Fifty one consecutive children with functionally univentricular heart prior to BDCPA were studied. All patients underwent cardiac catheterization and BNP measurement. Ventricular end-systolic wall stress (ESWS) and end-diastolic wall stress (EDWS) were calculated from cardiac catheterization data., Results: Median age was 1.1 years and 34% were female. Median BNP concentration was 90.4 pg/ml. Patients with high BNP (≥ 100 pg/ml) had higher pulmonary to systemic flow ratio (p = 0.014), a greater end-diastolic volume (p=0.009), more severe atrioventricular valve regurgitation (p= 0.02) and lower ventricular mass to end-diastolic volume ratio (p=0.006). BNP levels strongly related to EDWS (r = 0.75, p< 0.0001) and ESWS (r = 0.63, p < 0.0001). During median follow-up period of 3.2 years, 15 patients died and one underwent heart transplantation for refractory heart failure. On multivariate Cox regression analysis, high BNP concentration was an independent predictor of death or transplantation (HR 3.05, CI: 1.06-8.83, p = 0.04)., Conclusions: High BNP concentration at the first palliative stage towards Fontan circulation reflects high wall stress due to high volume load and insufficient ventricular hypertrophy. Moreover, high BNP levels at this stage were independently related to long-term outcome. BNP may be used as a guide to identify patients with high workload due to inadequate adaptation to hemodynamic load, who are at high risk., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Objective: The extracardiac conduit procedure is widely used for patients with heterotaxy syndrome with complex systemic and pulmonary venous anomalies; however, it lacks conduit-growth potential and requires long-term anticoagulation. We present the intracardiac Fontan procedure, which eliminates the above-mentioned disadvantages., Patients and Methods: Twenty-four patients (mean age, 4.1 years; weight, 13.0 kg) with heterotaxy syndrome underwent intracardiac Fontan operations between March 1995 and March 2008. In each patient, the anomalous systemic venous return with the isolated hepatic vein was redirected to the pulmonary artery using an intra-atrial baffle without obstructing the pulmonary venous pathway; this was accomplished by anterior (n=5), lateral (n=15) or posterior tunnel methods (n=4), depending on the anatomical relationship of the systemic and pulmonary venous pathways., Results: There was one (4%) in-hospital and two (8%) late deaths in total. Five (21%) patients underwent re-operation for either pulmonary venous obstruction or supraventricular tachycardia. The actuarial 12-year survival was 86% (the Kaplan-Meier survival plot). The freedom from re-operation at 10 years was 77%. Anticoagulation was not required except for two patients (8%) who had prosthetic valves and coagulation disorder. Obstruction of the systemic venous pathway was not observed in any patient; however, five (20%) patients had clinically significant postoperative arrhythmias. At the final follow-up, all survivors were categorised as the New York Heart Association class I., Conclusions: Aided by detailed preoperative anatomical and physiological diagnoses, intracardiac Fontan procedures were technically feasible in patients with complex systemic and pulmonary venous anomalies. The specific cardiac anatomy in these patients warranted this procedure; however, taking into consideration the improved outcomes of the modified Fontan procedure, this method should be performed with deliberation., (Copyright 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)

Objectives: We sought to provide a new method for quantifying collateral aortopulmonary flow in patients subsequent to construction of a bidirectional cavopulmonary shunt, and to clarify the clinical advantages of the new method., Methods: We performed lung perfusion scintigraphy and cardiac catheterization in 10 patients subsequent to construction of a bidirectional cavopulmonary shunt. First, the ratio of collateral to systemic flow was determined by whole-body images of lung perfusion scintigraphy, dividing the total lung count by the total body count minus the total lung count. Second, we integrated lung perfusion scintigraphy and cardiac catheterization data using a formula derived from the Fick principle, taking the ratio of pulmonary to systemic flow to be 1 plus the ratio calculated above and multiplied by the systemic saturation minus the inferior caval venous saturation divided by the pulmonary venous saturation minus the inferior caval venous saturation. Finally, the amount of collateral flow was obtained from the ratio of pulmonary to systemic flow. We evaluated the impact of collateral flow on the calculation of pulmonary vascular resistance., Results: The median age at bidirectional cavopulmonary shunt was 1.41 years, and the median age at catheterization was 2.33 years. The mean amount of collateral flow was 1.75 +/- 0.46 litres/min/m(2). The pulmonary vascular resistance calculated without considering the collateral flow was overestimated by an average of 57 +/- 23%, compared to the resistance calculated with our new method., Conclusions: The use of scintigraphy combined with catheterization allows accurate determination of aortopulmonary collateral flow, and avoids overestimation of pulmonary vascular resistance in these candidates for the Fontan circulation.

Purpose: The purpose of this study was to identify the risk factor of interim failure and to consider the method for detecting high-risk patients after Blalock-Taussing shunt., Methods: Between 1990 and 2004, 64 patients with functional single ventricle and low pulmonary flow underwent Blalock-Taussing shunt. We evaluated risk factors of interim failure by Logistic regression. Furthermore, assessments by catheterization and echocardiography were compared between Group Completion (Group C; 36 patients who had completed the Fontan operation or were Fontan candidates) and Group Failure (Group F; 20 patients who died before the Fontan operation or dropped out from Fontan candidate)., Results: In Group C, the survival at 10 years was 93%. The risk factor of interim failure was a systemic ventricular end-diastolic volume (SVEDV) of more than 250% of normal after shunt. The ventricular volume by echocardiography in Group C had been maintained between 190% and 200% of normal. By contrast, the ventricle in Group F had been significantly dilated after postshunt short term., Conclusions: The risk factor of interim failure was postshunt ventricular dilation, and the dilation was identified after postshunt short term. We recommend volume study within two to three months after shunt, and bidirectional cavopulmonary anastomosis should be immediately indicated to patients with ventricular dilation.

Intermediate results of the arterial switch operation for variety of cardiac anomaly with the transposed great arteries were studied to elucidate late results and problems. Seventy-five survivors of the arterial switch operation at our institute between 1991 and January 2007 were studied retrospectively. Mean follow-up time was 7.5 years. There were 2 late deaths, 3 reoperations, and 9 cases of catheter intervention, resulting in actuarial survival of 97%, and event-free rate of 83% at 16 years. Seventy patients (93%) were medication free and had no restriction on physical activities. Moderate pulmonary stenosis in 2 patients and mild pulmonary stenosis in 14 were observed. Moderate aortic regurgitation in 2 and mild aortic regurgitation in 20 were observed. Risk factors for right ventricular outflow obstruction were a diagnosis of double outlet right ventricle (DORV), association with aortic arch obstruction, and prior pulmonary artery banding (PAB). Risk factors for branch pulmonary artery stenosis (PS) were not clear; however, scar formation with use of xenograft patch and in-fold formation by redundant autologous pericardial patch for closure of the defects in the old aortic root seemed to be related with the stenosis. Risk factors for aortic regurgitation were age at operation and prior PAB. Early primary arterial switch operation may resolve these problems.

Recently, GATA4 and NKX2.5 were reported as the disease genes of atrial septal defect (ASD) but the relationship between the locations of their mutations and phenotypes is not clear. We analyzed GATA4 and NKX2.5 mutations in 16 familial ASD cases, including four probands with atrioventricular conduction disturbance (AV block) and two with pulmonary stenosis (PS), by PCR and direct sequencing, and examined their phenotypes clinically. Five mutations, including two GATA4 and three NKX2.5 mutations, were identified in 31.3% of the probands with ASD, and three of them were novel. The two GATA4 mutations in the probands without AV block were S52F and E359Xfs (c.1075delG) that was reported previously, and three NKX2.5 mutations in the probands with AV block were A88Xfs (c.262delG), R190C, and T178M. Additionally, we observed some remarkable phenotypes, i.e., dextrocardia with E359Xfs (c.1075delG) and cribriform type ASD with R190C, both of which are expected to be clues for further investigations. Furthermore, progressive, most severe AV block was closely related with a missense mutation in a homeodomain or with a nonsense/frame-shift mutation of NKX2.5 for which classification has not been clearly proposed. This pinpoints essential sites of NKX2.5 in the development of the conduction system., ((c) 2005 Wiley-Liss, Inc.)

Kawasaki disease (KD) is an acute systemic vasculitis syndrome of infants and young children. Although its etiology is largely unknown, epidemiological findings suggest that genetic factors play a role in the pathogenesis of KD. To identify genetic factors, affected sib-pair analysis has been performed. One of the identified peaks was located on the Xq26 region. A recent report of elevated expression of CD40 ligand (CD40L), which maps to Xq26, during the acute-phase KD, and its relationship to the development of coronary artery lesions (CAL) prompted us to screen for polymorphism of CD40L and to study the association of the gene to KD. A newly identified SNP in intron 4 (IVS4+121 A>G) is marginally over-represented in KD patients as compared to controls (109/602, 18.1 vs 111/737, 15.1%). When male KD patients with CAL were analyzed as a patient group, the SNP was significantly more frequent than in controls (15/58, 25.9%, vs 111/737, 15.1%, OR=2.0, 95% CI=1.07-3.66; P=0.030). Interestingly, this variation was extremely rare in a control Caucasian population (1/145, 0.7%). Our results suggest a role of CD40L in the pathogenesis of CAL and might explain the excess of males affected with KD.

It is rare to find multiple fistulas arising from all three coronary arteries and draining into the left ventricle. Coronary angiography revealed this anomaly in a one-year-old girl with tricuspid atresia after conversion to the Fontan circulation. To the best of our knowledge, this is the first report of such multiple fistulas in the setting of tricuspid atresia, and also the first report in childhood.

Objectives: Recent advances in medical and surgical treatment have led to the survival of increasing numbers of adults with congenital heart disease (CHD). However, the social status of these patients remains unknown. This survey investigated the social prospects for adults with CHD, and the limiting factors for social independence., Methods: A written questionnaire on patient characteristics, education, employability, marital status and insurability was designed to define the characteristics of social independence in adults with CHD. Randomly selected adults with CHD were enrolled: 13 patients with cyanotic unrepaired CHD (4 males, 9 females, mean age: 29.8 +/- 10 years, range: 18-56 years) and 102 patients with other CHDs (48 males, 54 females, mean age: 29.5 +/- 10 years, range: 18-74 years)., Results: University of California at Los Angeles functional class I-II was found in 94% of patients, medication in 46%, and hospitalization in 51%. Compared with the data from Japanese general population, study patients had a lower ratio of high school graduates (86% vs 94%), life insurability (51% vs 71%), marital status (31% vs 32%) and employability (82% vs 80%). Patients with unrepaired cyanotic CHD had significantly lower ratio than those with other CHDs (marital status 15%, p = 0.19; employability 40%, p = 0.0003; high school graduates 69%, p = 0.06; life insurability 18%, p = 0.02, respectively)., Conclusions: Factors affecting social independence in adults with CHD were severity of disease, continuing medication, lower level of education, lower self-esteem, and unknown natural history of CHD. To improve social independence in these patients, further development of medical and surgical therapy and more detailed knowledge of the patients, caretakers and society in this field are needed.

We report a two-year-old girl with asplenia, [A, L, L] DORV, pulmonary atresia, common AV valve, PDA, and TAPVC, who successfully underwent total cavo pulmonary connection (TCPC). Deep cyanosis was pointed out since birth. Cardiac catheterization performed on the sixth day after birth revealed a diminutive pulmonary artery tree of which PA index was 41 mm2/m2. Left modified Blalock-Taussig shunt was created at 27 days of age. The PA index increased to 282 mm2/mm2, but disparity in diameter between the left and the right pulmonary artery was yielded by PDA subsidence. Therefore additional contralateral B-T shunt was made at one year of age. Follow-up cardiac catheterization at 28 months of age showed well developed pulmonary artery; PA index of 460 mm2/m2, right pulmonary resistance (Rp) of 3.49 units, left Rp of 2.33 units, and estimated total Rp was 1.39. According to study, bidirectional Glenn procedure or TCPC was indicated. Considering necessity of urgent repair of common pulmonary vein obstruction, regurgitation of the common atrio-ventricular valve and pulmonary artery stenosis, TCPC was performed with concomitant repair of the associated lesions. Severe butterfly-figure stenosis of the central PA was augmented by anastomosing both the left SVC and the left-sided atrium. In conclusion, diminutive pulmonary artery could be adequately grown by phase-in Blalock-Taussig shunts. Pulmonary blood flow scintigraphy was thought to be useful for estimation of pulmonary resistance in such cases with different pulmonary resistance between right and left PA.

We report a six-year-old boy who underwent ascending aortic reconstruction for supravalvular aortic stenosis of diffuse type associated with Williams-Beuren syndrome. The diagnosis was first made at the age of six months. Because of progressive left ventricular hypertrophy, cardiac catheterization was performed at the age of five years and showed left ventricular pressure of 200 mmHg, the ascending aortic pressure of 202 mmHg, the descending aortic pressure of 115 mmHg, and left ventricular end-diastolic volume of 33.5 ml (90% of normal). Whole ascending aorta except sinus Valsalva was severely hypoplastic, so called diffuse type of supravalvular aortic stenosis. The ascending aorta was only 6.3 mm in diameter, whereas the diameter of the aortic annulus was 20.6 mm. The ascending aortic reconstruction of Doty's type was performed from the aortic root to the distal aortic arch with a equine pericardium reinforced by Dacron velour using selective perfusion to the right bracheocepharic artery, the left common carotid artery and the right femoral artery to avoid circulatory arrest. Postoperative course was uneventful and post operative catheterization revealed left ventricular pressure of 128 mmHg, the ascending aortic pressure of 126 mmHg and the descending aortic pressure of 90 mmHg. Mild residual pressure gradient was probably due to hypoplastic descending aorta. In conclusion, patch aortic reconstruction for ascending aorta under selective cerebral perfusion for a six-year old boy can be performed without postoperative neurological complication.

This study evaluated the clinical usefulness of analyzing left ventricular (LV) filling by color M-mode Doppler echocardiography in pediatric patients. The LV-filling patterns of color M-mode Doppler echocardiography were obtained by LV inflow in the apical 4-chamber or long-axis view, and the time difference between the occurrence of peak velocity at the mitral tip and in the apical region (M-AP) was calculated. The peak velocity at each depth was determined by adequate selection of the Nyquist limit by shifting the zero point after freezing the color M-mode. LV volume and posterior wall motion velocity were obtained simultaneously. The catheter-derived data were compared with echo-derived data in 7 patients (mean age 12.0 years). The M-AP correlated positively with the time constant of LV relaxation (tau; r = 0.83, p < 0.05), pulmonary capillary wedge pressure (r = 0.83, p < 0.05), and negatively with peak diastolic posterior wall motion velocity (r = -0.78, p < 0.05). The M-AP was compared with other echo-derived data between Group N (35 children with normal cardiac function, mean age 4.3 years) and Group F (12 children with LV ejection fraction less than 40%, mean age 9.5 years). The M-AP was significantly longer in Group F (53.3 +/- 14.0 vs 116.5 +/- 30.5 msec; p < 0.001), but there was no significant difference in the E/A or deceleration time of E between the 2 groups. In Group N, the E/A correlated to LV end-diastolic volume and heart rate, but the M-AP showed no correlation. In Group F, the M-AP correlated with the percentages of normal LV end-diastolic volume (r = 0.76, p < 0.01) and LV ejection fraction (r = -0.58, p < 0.05). The M-AP was not influenced by LV size or heart rate and could easily differentiate normal heart from failing heart, and thus this is a useful parameter for evaluating diastolic function in pediatric patients.

We reported a successful case of the modified Norwood operation for a 21-day-old neonate with hypoplastic left heart syndrome (MS and AS) associated with an aberrant right subclavian artery and a persistent left superior vena cava. The modified Norwood operation was performed without total circulatory arrest and Cardiac arrest. A 4 mm Gore-Tex graft, which was anastomosed between the right carotid artery and the right pulmonary artery for systemic-pulmonary shunt, was used for cerebral perfusion during aortic arch reconstruction. Coronary perfusion was performed with a small cannula placed on the relatively large ascending aorta during anastomosis between the main pulmonary artery and the ascending aorta. Equine pericardial patch was used for aortic arch reconstruction and the ascending aorta was directly anastomosed to a part of the main PA. Postoperative course was uneventful and postoperative MRI revealed no stenosis of the aortic arch and the pulmonary artery.

Konno's operation was successfully performed on a small infant, weighing 3.8 kg. She underwent pulmonary artery banding on the 29th day of life under the diagnosis of partial atrioventricular septal defect with a small left ventricle, associated with polysplenia. Intracardiac repair was subsequently performed on the 45th day of life, which included atrial partition and mitral valvuloplasty. Eleven months after the second operation, wedge resection on the narrow subaortic fibrous lesion was done concomitant with mitral revalvuloplasty. However, repeated echocardiac study disclosed progression of subaortic stenosis. Cardiac catheterization performed at 2 years of age showed the pressure gradinet of 90 mmHg between the aorta and the LV. The aortic annulus and the subaortic lesion measured 10 mm and 4 mm in diameter respectively. Severe subaortic stenosis was created by protrusion of abnormal mitral tissue to the LV outflow, in addition by secondary circumferential fibrous tissue growth and hypertrophy of the ventricular septum. Konno's operation using on 18A Carbomedics valve was chosen for the certain relief of the subaortic stenosis. Although the operation required extensive ventriculotomy and septostomy because of the small aortic annulus, the postoperative course was uneventful. Two months after the operation echocardiography showed good LV function and low RV pressure of about 40 mmHg.

We report a case of D-transposition of the great arteries associated with double aortic arch. An aortic root angiogram revealed that the left-sided aortic arch was anterior and smaller than the right one and the descending thoracic aorta located on the left side of the spine. Balloon atrial septostomy was performed at one day of age. The patient underwent a simultaneous arterial switch procedure and division of the vascular ring at the isthmus of the left aortic arch through a median sternotomy incision at the age of 16 days. There was persistent postoperative difficulty in weaning the patient from the ventilator. Magnetic resonance images showed re-formation of pseudovascular ring by the connective tissue grown around the divided arch. At the age of 23 days, resection of the remnant of left aortic arch including the left subclavian artery and the diverticulum concomitant with vascular suspension procedure was performed through a left lateral thoracotomy. The patient was subsequently extubated without difficulty and was discharged from the hospital. It is though that a vascular suspension procedure and resection of the subclavian artery are necessary to avoid respiratory obstruction when the great arteries are in an anteroposterior position and Lecompte procedure is performed.

We report an alternative method of repairing partial anomalous pulmonary venous return (PAPVR) wherein anomalous veins drain into the high portion of superior vena cava (SVC). A six year old girl, weighing 19 kg underwent cardiac catheterization for cardiomegaly. The study showed sinus venous defect and pulmonary veins anomalously drained from the right upper and middle lobe into the high SVC. Under cardiopulmonary bypass with aortic and bicaval direct cannulation, a 2-cm-wide pedicled flap made from the middle part of the right atrium (RA), was pulled into the SVC. The atrial flap divided the SVC from the ostium of the uppermost pulmonary vein (PV) to the ASD. This posterior tunnel drained anomalous PV blood into the left atrium. Postoperative echocardiogram and magnetic resonance imaging showed adequate space in the SVC and the PV channel. The patient maintained normal sinus rhythm after surgery. The advantage of this method is to minimize the risk of injuring the sinus node or sinus node artery, and to easily reconstruct both the SVC and PV channel without using prosthetic materials, even in patients with small RA.

Eight consecutive patients with tetralogy of Fallot associated with pulmonary atresia successfully underwent corrective surgery without using an extracardiac conduit at a mean age of 2 years 4 months. Each of these patients had undergone a classical or modified Blalock-Taussig shunt at a mean age of 55 days. In 6 cases, central PA was fully mobilized and pulled forward. An autologous flap was made from the rudimentary PA trunk or right PA, then it was anastomosed to the endocardium of RV infundibulum as a posterior wall of the outflow tract. In 7 cases, concomitant extensive pulmonary angioplasty was performed on stenotic and under-developed lesions which were caused by tissue contraction of the PDA. During follow up period from 11 to 25 months, all patients have remained in good condition without restenosis of the RVOT. The mean pressure ratio of RV over LV measured 0.42 in 5 patients who underwent catheterization after operation. We conclude that these procedures are useful for early corrective surgery of tetralogy of Fallot with pulmonary atresia.

This study assessed the validity of cine magnetic resonance imaging (MRI) for measuring right and left ventricular volumes by using Simpson's rule in children with complex congenital heart disease. Forty-five patients with complex congenital heart disease (average age 2.6 years) and 10 controls (average age 2.3 years) were evaluated. The whole heart was encompassed by contiguous transverse sections. Ventricular volumes were calculated by adding luminal areas determined in each section at end diastole and end systole. End-diastolic and end-systolic volumes by MRI in both groups correlated well with those by ventriculography (r>0.89). Comparison of the ejection fraction in both ventricles in both groups yielded a good correlation between MRI and ventriculography (r>0.67). MRI technique in both groups had low intraobserver and interobserver variation (<6%). Cine MRI provides a suitable noninvasive means of quantifying ventricular volume in children with complex congenital heart disease.

Background: We report on 10 patients with Kawasaki disease and thrombocytopenia who were found to have a high incidence of coronary artery aneurysm and acute myocardial infarction. The clinical features of these patients, the cause of their thrombocytopenia and the relationship between cardiovascular pathology and thrombocytopenia were analyzed., Methods: The clinical features of 10 patients with Kawasaki disease found to have thrombocytopenia (group A: mean age 8.0 +/- 7.0 months) and those of 293 patients with Kawasaki disease (group B, controls: mean age 13 +/- 20 months) were analyzed. Coagulation studies and bone marrow aspiration were performed to ascertain the cause of the thrombocytopenia in nine out of 10 subjects in group A., Results: The minimum platelet count was 4-12 x 10(4)/mm3 (average day of illness, 10.3) and platelet counts were elevated to the baseline value within 1-2 weeks of onset of the illness. Low fibrinogen concentrations, high levels of fibrin degradation products, and low erythrocyte sedimentation rates with high C-reactive protein levels were observed in seven patients. In two other patients, immature megakaryocytes with normal coagulation values were observed. The differences in the incidence of coronary artery aneurysm and acute myocardial infarction between groups A and B were highly significant (coronary artery aneurysm: 60% in group A, 8.9% in group B; acute myocardial infarction: 40% in group A, 0.3% in group B)., Conclusions: In many patients with Kawasaki disease and thrombocytopenia, the thrombocytopenia appears to be a result of intravascular coagulation, and to be one of the risk factors for acute myocardial infarction.

Objectives: This study assessed the usefulness and safety of magnetic resonance imaging (MRI) for systematically diagnosing heterotaxia in infants., Background: Although it is important to diagnose and treat infants with heterotaxia, which is associated with viscerobronchial cardiovascular anomalies, systematic diagnosis of these anomalies by a single imaging technique is difficult., Methods: Twenty patients with heterotaxia were evaluated. The infants ranged in age from 21 days to 12 months (average 5.2 months, average body weight 4.3 kg). Electrocardiographically gated MRI was performed by spin echo imaging techniques operating at 0.5 tesla., Results: In all 20 patients, MRI results were sufficient to evaluate these anomalies without serious complications. In 17 patients, neither a spleen nor splenules were detected, but in 3 patients, a polymorphous spleen was visualized. In all 20 patients, bronchial anatomies were clearly visualized (bilateral eparterial bronchi in 14 patients, bilateral hyparterial bronchi in 2 and normal bronchial patterns in 4). Additionally, in a comparison of 149 observations of cardiovascular anatomy by MRI with those by angiography, discrepancies were found in only 10 observations (6.7%)., Conclusions: Magnetic resonance imaging was found to be safe and very useful for the systematic diagnosis of heterotaxia in infants.

Recently, pulmonary arterial banding (PAB) and the Blalock-Taussig shunting operation (B-T) have been performed as a preparatory procedure for Jatene's operation for patients with complete transposition of the great arteries (TGA) without patent ductus arteriosus (PDA) or ventricular septal defect (VSD). We reviewed the echocardiographic findings of 29 patients after PAB and B-T. Four cases had abnormal catheterization data after PAB and B-T (Group 1). Twenty-five control cases with successful two-stage Jatene's operation exhibited normal data (Group 2). We studied 1) the grade of distortion of the interventricular septum as the a/b ratio on two-dimensional echocardiogram (2-DE), 2) the left ventricular (LV) shortening fraction (LVSF), 3) the thickening fraction (ThF), 4) the LV posterior wall thickness in diastole (LVPWTd) on M-mode echocardiogram, and 5) the LV inflow tract (LVIT) flow pattern on pulsed Doppler echocardiogram. In Group 1, all cases revealed LVSF less than 0.19 (control group: 0.40-0.56), ThF less than 0.18 (control group: 0.43-0.81), the a/b ratio higher than 0.88 (control group: 0.53-0.79), and LVPWTd thicker than 0.67 cm (control group: 0.45-0.59 cm). These data are markedly deviated from the range of the control data (p < 0.01). All cases in Group 1 had LVIT flow lacking the rapid filling phase, which was recognized in the control group. These abnormal echocardiographic findings suggest LV failure in adaptation against excessive after-load due to very tight PAB, and these figures are very different from the control group. These unusual postoperative cardiac abnormalities can be detected noninvasively using 2-DE and Doppler echocardiography.(ABSTRACT TRUNCATED AT 250 WORDS)

A 3-month-old boy, mildly cyanosed and tachypneic, was found by cineangiography to have a nonbranching main pulmonary artery arising from the right ventricle and connecting to the descending aorta via a large persistent ductus arteriosus. The left and right pulmonary arteries arose as a common "trunk," before branching, from the ascending aorta. Complete surgical correction was performed at 7-months of age. Subsequent cardiac catheterization has demonstrated normal pressures and blood oxygen saturation in all right-heart chambers and the great arteries.

Identification of the azygos or hemiazygos connection is important in the diagnosis of congenital heart disease, especially in cases of the polysplenia syndrome. Although two-dimensional echocardiographic observation of this anomaly has been reported, the direct visualization of drainage sites is still rather difficult. A method for visualizing the azygos or hemiazygos connection by a combined use of two-dimensional and pulsed and color Doppler echocardiography was reported here. Subjects consisted of 10 patients with the polysplenia syndrome, each of whom had been confirmed as having the azygos or hemiazygos connection; five each of the azygos and hemiazygos types. The transducer was placed over the second intercostal space on the right side for the azygos, and on the left side for hemiazygos connection. The two-dimensional echocardiographic plane was adjusted to visualize the long-axis view of the superior vena cava; the right superior vena cava for the azygos and the left superior vena cava for the hemiazygos connection. The mode of the equipment was changed to color Doppler, and an orange-colored blood flow toward the transducer, draining into the superior vena cava was carefully sought. Finally the pulsed Doppler mode was selected and the sampling volume was set in the orange-colored area. The wave form of the pulsed Doppler echocardiogram of the azygos or hemiazygos vein potentially had two peaks per cardiac cycle, and was modified by respiration. We found azygos or hemiazygos veins coursing from the posterior aspect of the right pulmonary artery in seven of 10 cases by two-dimensional echocardiography alone, while we observed all veins by the additional use of color and pulsed Doppler echocardiography.