Comparison between transcatheter versus surgical intervention for pediatric aortic valvular stenosis: a multicenter study in Japan.

It is controversial whether children with isolated aortic valvular stenosis (vAS) initially undergo transcatheter or surgical aortic valvuloplasty (BAV or SAV). This multicenter retrospective case-control study aimed to explore outcomes after BAV or SAV for pediatric vAS. We studied children (aged < 15 years) with vAS treated at 4 tertiary congenital heart centers, and compared the rates of survival, reintervention, and valve replacement between patients with BAV and SAV. A total of 73 subjects (BAV: N = 52, SAV: N = 21) were studied. Age and aortic annulus z-score at the first presentation were 85 (26-530) days and - 0.45 (- 1.51-0.59), respectively. During the follow-up period of 121 (47-185) months, rates of 10-year survival (BAV: 88% vs. SAV: 92%, P = 0.477), reintervention (BAV: 58% vs. SAV: 31%, P = 0.626), and prosthetic/autograft valve replacement (BAV: 21% vs. SAV: 19%, P = 0.563) did not differ between the groups. Freedom from reintervention rate significantly correlated with aortic annulus z-score (hazard ratio [HR] 0.66, 95% confidence interval [CI] 0.49-0.88, P = 0.005), and freedom from prosthetic/autograft valve replacement rate significantly correlated to the degree of aortic regurgitation after the first intervention (HR: 4.58, 95% CI 1.19-17.71, P = 0.027). Propensity score-matched analysis (N = 16) did not show the differences in survival and reintervention rates between the groups. Long-term survival was acceptable, and the rates of freedom from reintervention and prosthetic/autograft valve replacement were comparable between children with vAS who underwent BAV and SAV.

Transcatheter atrial septal defect closure late after completion of biventricular circulation in patients with pulmonary atresia intact ventricular septum or critical pulmonary stenosis.

OBJECTIVE: This study aimed to explore anatomical and hemodynamic features of atrial septal defect, which was treated by transcatheter device closure late after completion of biventricular circulation in patients with pulmonary atresia and intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS). METHODS: We studied echocardiographic and cardiac catheterization data, including defect size, retroaortic rim length, single or multiple defects, the presence of malalignment atrial septum, tricuspid and pulmonary valve diameters, and cardiac chamber sizes, in patients with PAIVS/CPS who underwent transcatheter closure of atrial septal defect (TCASD), and compared to control subjects. RESULTS: A total of 173 patients with atrial septal defect, including 8 patients with PAIVS/CPS, underwent TCASD. Age and weight at TCASD were 17.3 ± 18.3 years and 36.6 ± 13.9 kg, respectively. There was no significant difference in defect size (13.7 ± 4.0 vs. 15.6 ± 5.2 mm, p = 0.317) and the retro-aortic rim length (3.7 ± 4.3 vs. 3.6 ± 0.3.1 mm, p = 0.948) between the groups; however, multiple defects (50% vs. 5%, p < 0.001) and malalignment atrial septum (62% vs. 14%. p < 0.001) were significantly frequent in patients with PAIVS/CPS compared to control subjects. The ratio of pulmonary to systemic blood flow in patients with PAIVS/CPS was significantly lower than that in the control patients (1.2 ± 0.4 vs. 2.0 ± 0.7, p < 0.001); however, four out of eight patients with atrial septal defect associated with PAIVS/CPS had right-to-left shunt through a defect, who were evaluated by the balloon occlusion test before TCASD. The indexed right atrial and ventricular areas, the right ventricular systolic pressure, and mean pulmonary arterial pressure did not differ between the groups. After TCASD, the right ventricular end-diastolic area remained unchanged in patients with PAIVS/CPS, whereas it significantly decreased in control subjects. CONCLUSIONS: Atrial septal defect associated with PAIVS/CPS had more complex anatomy, which would be a risk for device closure. Hemodynamics should be individually evaluated to determine the indication for TCASD because PAIVS/CPS encompassed anatomical heterogeneity of the entire right heart.

Coronary anomalies associated with ventricular septal defect.

Coronary anomalies encompass different conditions in terms of anomalous origin, coursing, and branching, which are occasionally associated with congenital heart disease. This study aimed to explore coronary anomalies associated with ventricular septal defect that was a stereotypical congenital heart disease. We retrospectively reviewed angiographic findings in patients with ventricular septal defect who required corrective surgery, and identified coronary abnormalities, including anomalous origin, coursing, and branching of coronary arteries based on angiography. We studied the prevalence and types of coronary anomalies among them. A total of 998 patients with ventricular septal defect was studied. Age and weight were 2.0 (2.1-15.2) months and 5.7 (4.1-8.7) kg, respectively. There were 115 patients (12%) with syndrome or genetic disorder, and 34 patients (3%) with extracardiac major organ anomalies. The overall prevalence of coronary anomalies was 6.2%. All coronary anomalies were anomalous origin and coursing, among 3 patients accompanied anomalous intrinsic coronary anatomy. Originating within aortic root above the Valsalva sinus was the most common coronary anomaly. Coronary anomalies were independently correlated with bicuspid aortic valve (odds ratio [OR]: 8.02, 95% confidence interval [CI]: 2.34-23.4) and persistent left superior caval vein (OR: 5.02, 95% CI: 1.93-11.7). We showed the possibility that minor cardiac variants, such as bicuspid aortic valve and persistent left superior caval vein, contribute to higher prevalence of coronary anomalies in patients with ventricular septal defect.

Home-inhaled nitric oxide in a child with pulmonary arterial hypertension associated with post-operative pulmonary venous obstruction.

Home-inhaled nitric oxide therapy was effective and feasible in the patients with intractable pulmonary arterial hypertension. We present the case of a child with severe pulmonary arterial hypertension associated with post-operative pulmonary venous obstruction who was treated with home-inhaled nitric oxide therapy.

Pulmonary vascular resistance and compliance in individuals with trisomy 18.

Individuals with trisomy 18 (T18) usually have congenital heart disease, often with pulmonary hypertension, which is associated with poor outcomes. This study aimed to explore the characteristics of pulmonary circulation including pulmonary vascular resistance (Rp) and compliance (Cp) among them. We retrospectively reviewed cardiac catheterization data in subjects with T18, trisomy 21 (T21), and without chromosomal anomaly (control group) who were referred due to heart failure associated with ventricular septal defect between 2000 and 2020. Pulmonary hemodynamic parameters including Rp and Cp were compared between these groups. We studied 20 subjects with T18, 88 subjects with T21, and 240 control subjects. There was no significant difference in age (T18: 4.6 [3.0-6. 9] vs. T21: 2.8 [1.9-4.0] vs. control: 2.9 [1.6-3.2] months, p = 0.06) and mean pulmonary arterial pressure (T18: 41 [33-49] vs. T21: 35 [30-41] vs. control: 36 [28-43] mmHg, p = 0.121) between the groups. The pulmonary to systemic blood flow ratio (Qp/Qs) (p = 0.983), Rp (p = 0.449), and Cp (p = 0.195) did not differ between T18 and control groups. However, Qp/Qs and Cp in T18 group were significantly greater than that in T21 group (T18: Qp/Qs: 3.4 [2.3-5.2] vs. T: 21 2.3 [1.7-3.7], p = 0.001. Cp: 3.5 [2.3-5.5] vs. 2.3 [1.6-3.1] mmHg/mL/m2 , p = 0.007), while Rp was identical between the groups (T18: 2.0 [1.6-3.3] vs. T21: 2.3 [1.7-3.7], p = 0.386). The pulmonary circulation in T18 subjects differed from that observed in T21 subjects, and identical to that observed in control subjects. Pulmonary hypertension is expected to be normalized after reasonable corrective surgery in T18 patients with congenital heart disease.

Pulmonary vascular resistance and compliance in pulmonary blood flow alterations in children with congenital heart disease.

There is a unique hyperbolic relationship between pulmonary vascular resistance (Rp) and compliance (Cp); however, the characteristics of this coupling curve in pulmonary blood flow alterations remains unknown in children with congenital heart disease. We aimed to explore the Rp-Cp coupling and resistant-compliance (RC) time among them. We retrospectively reviewed catheterization data and calculated Rp and Cp in 217 subjects with ventricular septal defect. Median age and weight at catheterization were 2.8 (1.7-4.4) months and 4.3 (3.7-5.3) kg, respectively. Pulmonary hemodynamic parameters were as follows: mean pulmonary arterial pressure: 36 (28-43) mmHg; the amount of pulmonary blood flow (Qp): 14.2 (11.6-17.6) L/min/m2; Rp: 1.95 (1.38-2.59) Wood unit m2; Cp: 2.98 (2.42-3.88) mmHg/mL/m2; and RC time: 0.35 (0.30-0.40) s. RC time remained unchanged according to alterations in Qp (P = 0.206); however, the relationship between logarithm transformations of Rp and Cp showed more steeper according to an increase in Qp. The pulmonary circulation depends upon Cp rather than Rp according to the degree of Qp despite the constancy in RC time. We should take both Rp and Cp into consideration when evaluating the pulmonary circulation among children with congenital heart disease.

Coronary arterial dominance in patients with congenital heart disease.

Coronary arterial dominance is concerned in the management of ischemic heart disease. In particular, right coronary arterial dominance is having a risk for three-vessel coronary artery disease. Thus, this study aimed to explore coronary arterial dominance in patients with congenital heart disease. The study involved 250 patients, of which 105 patients were with tetralogy of Fallot (TOF), 100 patients with ventricular septal defect (VSD), and 45 patients with Kawasaki disease (KD). We retrospectively reviewed their ascending aortography to determine their coronary arterial dominance, Z-scores of coronary artery diameter, and the ascending aortic curvature, which pertained to the angle between the aortic annulus plane and ascending aortic plane. We identified relevant factors that contribute to having right coronary arterial dominance. Age and weight of the 250 subjects were 2.9 (1.0-8.7) months and 7.7 (5.0-9.4) kg, respectively. The Z-scores of right coronary and anterior descending arteries significantly differed among patients with TOF, VSD, and KD (P < 0.001, P = 0.001). However, there were no significant differences in the Z-scores of left main trunk and circumflex arteries. Right coronary arterial dominance occurred in 89%, 49%, and 61% in patients with TOF, VSD, and KD, respectively (P < 0.001). The presence of TOF was the most powerful predictor for right coronary arterial dominance (odds ratio: 10.31, 95% confidence interval: 4.11-27.2, P < 0.001). We found the robust relationship between right coronary arterial dominance and TOF. Patients with TOF may have an increased risk for the development of coronary artery disease during adulthood.

Distensibility of the ductus arteriosus in neonates and young infants undergoing transcatheter closure.

Device migration is one of serious complications in neonates and infants undergoing transcatheter closure of the patent ductus arteriosus (PDA). We hypothesize that neonates and young infants possess the distensibility of the ductus, which may be related to device migration. We retrospectively reviewed angiographic findings in 41 neonates and infants who underwent transcatheter closure of PDA. We measured diameters of the ductus at the pulmonary (PA) side, the center, and the aortic (AO) side before PDA closure, and the device center diameter after device closure. The distensibility index was defined as the ratio of the device center diameter after device deployment to the diameter at the center of the ductus before PDA closure. Age and weight at the procedure were 168 (117-260) days and 5.3 (4.3-6.9) kg, respectively. Thirty-seven subjects accomplished the successful device closure, and four subjects were declined because of the device instability or migration. Implanted devices included Amplatzer Duct Occluders in 33 subjects and Amplatzer Vascular Plug-2 in 8 subjects. The PDA diameters at PA side, at the center, AO side, and the device center diameter were 3.2 (2.2-4.3) mm, 4.7 (3.6-5.7) mm, 7.7 (6.3-9.4) mm, and 5.8 (4.2-6.9) mm, respectively. The PDA diameter before closure was not correlated age and weight. The distensibility index was 1.28 (1.06-1.64), which was significantly correlated to age (r = - 0.49, P = 0.001) and weight (r = - 0.53, P < 0.001). Infants with the younger age and the lower weight have the more distensible PDA, which may be a risk for device migration.

Ventricular Repolarization Dispersion is a Potential Risk for the Development of Life-Threatening Arrhythmia in Children with Hypertrophic Cardiomyopathy.

The risk stratification and early interventions are necessary in patients with hypertrophic cardiomyopathy (HCM), as life-threatening arrhythmia (LTA) is a leading cause of death. This study aimed to explore whether an interval between the peak of the T wave to the end terminal of the T wave (Tp-e), which represents ventricular repolarization dispersion, could predict the risk for LTA in children with HCM. We analyzed electrocardiography at the first and last visits in children (aged < 15 years) with HCM, and compared Tp-e interval and the ratio of Tp-e interval to QT interval (Tp-e/QT) between children with and without LTA. We studied 25 children with HCM. During the follow-up of 85 (38-146) months, there were 7 children with LTA. The 5-year sudden cardiac death (SCD) risk was 1.4 (1.1-2.5) %, which suggested that our cohort consisted of patients at a lower risk for SCD. Age was significantly older in children with LTA compared to those without it (12.5 vs.1.0 years, P = 0.037), although sex, the presence of family history and symptoms at diagnosis, the maximum left ventricular wall thickness Z-score did not differ between the groups. At the last electrocardiography before LTA, corrected Tp-e interval and Tp-e/QT ratio were significantly greater in patients with LTA compared to those in patients without LTA (corrected Tp-e: 103 vs. 78 ms, P = 0.020; Tp-e/QT: 0.28 vs. 0.22, P = 0.046). Corrected Tp-e and Tp-e/QT ratio cutoff values of 91 ms and 0.28 yielded as the predictors for LTA with sensitivity of 85% and 72%, specificity of 71% and 89%, respectively. Prolonged absolute and corrected Tp-e intervals and an increase in the Tp-e/QT ratio can be useful predictors for LTA in children with HCM. We offer temporal assessments of ventricular repolarization dispersion to stratify the risk for the development of LTA/SCD among children with HCM.

Long-Term Outcomes After an Individualized Strategy in Patients with Pulmonary Atresia and Intact Ventricular Septum.

This retrospective cohort study aimed to explore the long-term outcomes of an individualized strategy in patients with pulmonary atresia and intact ventricular septum (PAIVS). We analyzed survival and reintervention rates and identified risk factors for outcomes in patients with PAIVS treated based on individual right heart structures between 1979 and 2019. Ninety-five patients were included in this study. The z-scores of the pulmonary annulus, tricuspid annulus, and right ventricular end-diastolic volume were - 3.30 (- 15.15 to 1.83), - 0.70 (- 4.65 to 2.33), and - 1.51 (- 6.35 to 1.18), respectively. Right ventricular-dependent coronary circulation occurred in 15% of the patients. Among the 63 patients attempting biventricular strategy at first, 55 patients achieved biventricular circulation, 3 patients had one-and-a-half circulation, and 4 patients died perioperatively. Among the 33 patients attempting univentricular strategy at first, 10 patients died before the completion of Fontan operation, 17 patients (48%) accomplished Fontan operation, and 5 patients waited for Fontan operation. In one patient, conversion to biventricular circulation occurred. During the follow-up period of 720 person-years, the 20-year survival rate was significantly higher in patients with biventricular circulation than in those patients with univentricular circulation (93% vs. 67%, P < 0.001). Freedom from reintervention rates at 20 years was significantly lower in patients with biventricular circulation than in those patients with univentricular circulation (29% vs. 72%, P < 0.001). The pulmonary annulus z-score was an independent risk factor for reintervention in patients with biventricular circulation. Patients with biventricular circulation had an acceptable survival rate, but a high reintervention rate. Meanwhile, patients with univentricular circulation had high mortality before the completion of Fontan operation, although the reintervention rate was relatively low.

Home High-Flow Nasal Cannula Therapy in Children with Congenital Heart Disease.

High-flow nasal cannula (HFNC) therapy has been applied in the perioperative respiratory care for children with congenital heart disease and respiratory problems. However, the information about the feasibility of home HFNC therapy remains lacking among them. We retrospectively reviewed 5 children with congenital heart disease and respiratory problems who underwent home HFNC therapy, and evaluated their feasibility and safety. Age and weight at the introduction of home HFNC therapy were 19 (2-119) months and 5.3 (3.1-11.4) kg, respectively. All subjects had chromosomal anomaly including trisomy 18 in 3 and trisomy 21 in 2 subjects. Cardiac diagnoses included ventricular septal defect in 3, tetralogy of Fallot with complete atrioventricular septal defect in one, and pulmonary atresia with ventricular septal defect in another subject. Other comorbidities involved pulmonary hypertension in 4, micrognathia in 4, West syndrome in one, and bronchial asthma in one subject. Respiratory manifestations involved cyanosis due to upper airway obstruction in 2 and central hypopnea in 2, and recurrent pneumonia in one subject. After home HFNC therapy, systemic oxygen saturation significantly increased from 60 (40-78)% to 83 (83-96)% (P = 0.04), while heart rate and blood partial pressure of carbon dioxide were significantly decreased. There was no adverse event relevant to home HFNC during the follow-up period of 12 (5-49) months. Among them, one patient subsequently underwent tracheotomy at 11 years of age, and two patients weaned to conventional home oxygen therapy at 7 and 23 months of age. Home HFNC is safe and feasible in children with congenital heart disease and respiratory problems.

Characteristics of the pulmonary circulation in infants with complete atrioventricular septal defect.

OBJECTIVE: Infants with complete atrioventricular septal defect occasionally accompany pulmonary hypertension; however, the pulmonary circulation can be altered by pulmonary vascular conditions as well as the left heart lesions. This study aimed to explore whether the left heart lesions were related to the pulmonary circulation among them. METHODS: We performed echocardiography and cardiac catheterisation in 42 infants with complete atrioventricular septal defect and studied relationships between the pulmonary haemodynamic parameters and the left heart morphology. RESULTS: Age and weight at preoperative evaluation were 65 days (47-114) (the median following interquartile range) and 5.5 kg (4.0-7.1), respectively. There were 27 individuals with Down syndrome. Gestational age was 38 weeks (37-39). Catheterisation showed mean pulmonary arterial pressure: 36 (29-46) mmHg, the ratio of pulmonary to systemic blood flow: 3.45 (2.79-4.98), pulmonary vascular resistance: 2.20 Wood units·m2 (1.53-3.65), and pulmonary arterial compliance: 2.78 (1.86-4.10) ml/Hg/m2. Echocardiography showed the Rastelli classification type A in 28 and type C in 14, moderate or severe left atrioventricular valve regurgitation in 19 patients (45%), atrioventricular valve index of 0.67 (0.56-0.79), left ventricular end-diastolic volume z score of 4.46 (1.96-7.78), and aortic valve diameter z score of -0.70 (-1.91 to 0.20). Multivariable regression analysis revealed that preoperative pulmonary vascular resistance was significantly correlated to gestational age (p = 0.002), and that preoperative pulmonary arterial compliance was significantly correlated to gestational age (p = 0.009) and Down syndrome (p = 0.036). CONCLUSIONS: The pulmonary circulation does not depend upon the presence of left heart lesions but gestational age and Down syndrome in infants with complete atrioventricular septal defect.

Echocardiographic Assessments for Peripartum Cardiac Events in Pregnant Women with Low-Risk Congenital Heart Disease.

This retrospective cohort study aimed to explore the relationship between temporal changes in the cardiac function and peripartum cardiac events in pregnant women with low-risk congenital heart disease.We performed echocardiography at early and late pregnancy and postpartum in 76 pregnant women with low-risk congenital heart disease, and compared echocardiographic parameters between subjects with and without peripartum cardiac events. Median age at delivery was 27 (range, 24-31) years. The ZAHARA and CARPREG II scores suggested that most women were found to be at low-risk for pregnancy. Fifteen subjects had cardiac events that included heart failure in 10, arrhythmia in 4, and pulmonary hypertension in one subject. The left ventricular and atrial volumes significantly increased from early pregnancy toward late pregnancy, and the E/A ratio and global longitudinal strain significantly decreased from early pregnancy toward late pregnancy. The left atrial volume (67 [53-79] versus 45 [35-55] mL, P = 0.002) and plasma brain natriuretic peptide level (58 [36-123] versus 34 [18-48] pg/mL, P = 0.026) at late pregnancy were significantly higher in subjects with cardiac events than in those without cardiac events.An increase in the left atrial volume followed by mild left ventricular diastolic dysfunction is related to peripartum cardiac events in women with congenital heart disease who are at low risk for cardiac events during pregnancy.

Headache and Left Ventricular Efficiency After Transcatheter Closure of Atrial Septal Defect.

OBJECTIVE/BACKGROUND: This study aimed to clarify the relationship between migraine-like headache and ventriculo-arterial coupling after transcatheter closure of the atrial septal defect in children. We hypothesized that migraine headache after defect closure would be related to an abnormal hemodynamic response against an increased left ventricular filling. DESIGN: A retrospective cohort study. METHODS: We calculated the end-ventricular systolic elastance (Ees), effective arterial elastance (Ea), and ventricular energy efficiency approximated based on echocardiography before and after defect closure, and compared these parameters between the subjects with and without headache after defect closure. RESULTS: A total of 167 subjects were studied. Age at the procedure, defect diameter, and pulmonary to systemic blood flow ratio were 11 (9-17) years, 12.8 (9.2-16.0) mm, and 1.8 (1.6-2.3), respectively. We identified 47 (28%) subjects with migraine headache after defect closure. Although there was no significant difference in the Ees, Ea, and ventricular energy efficiency before defect closure between the groups, the Ees (4.0 [3.4-4.9] vs 4.8 [3.7-6.1], P = .014) and ventricular energy efficiency (0.79 [0.76-0.82] vs 0.83 [0.79-0.85], P = .001) after defect closure in subjects with headache were significantly lower than those in subjects without headache. Migraine headache after defect closure was significantly associated with age (odds ratio: 0.97, 95% confidential interval: 0.94-1.00, P = .036) and a decrease in the ventricular energy efficiency after defect closure (odds ratio: 6.42, 95% confidential interval: 2.76-14.90, P < .001). CONCLUSION: A loss of ventricular energy efficiency was common in pediatric subjects with migraine-like headache after transcatheter closure of the atrial septal defect, which suggested that the left ventricular function maladaptation was related to headache development after defect closure. We advocate that an impaired ventriculo-arterial coupling may be one of the mechanisms for developing attacks in not only this population but also in other patients with migraine.

Early palliative balloon pulmonary valvuloplasty in neonates and young infants with tetralogy of Fallot.

We aim to clarify the efficacy of early palliative balloon pulmonary valvuloplasty (BPV) in neonates and young infants (< 60 days) with tetralogy of Fallot (TOF). We performed palliative BPV in 31 subjects, regardless of the presence of cyanosis, with Z score of the pulmonary valve diameter (PVD) less than - 2.00. Primary and secondary endpoints were to avoid early surgical interventions for subjects within 6 months of age and to undergo the pulmonary valve-sparing procedure at corrective surgery, respectively. We studied factors associated with these outcomes among them. BPV was performed at 19 days (14-33) of age and with a weight of 3.34 kg (3.02-3.65). Systemic oxygen saturation, Z score of the PVD, and pulmonary arterial index (PAI) were 87% (81-91), - 3.56 (- 4.15 to - 2.62), and 128 mm2/m2 (102-157), respectively. There were 16 and 13 subjects who avoided early surgical interventions and transannular repair, respectively. At the primary endpoint, there was no significant difference in age, weight, systemic oxygen saturation, and Z score of the PVD and PAI between the groups. However, there was a significant difference in the infundibular morphology (severe: mild-to-moderate, 8:8 vs 13:2, P = 0.029) between the groups. We performed prophylactic BPV within 30 days after birth in 7 acyanotic TOF patients with severe infundibular obstruction, among whom 5 avoided early surgical intervention. At the secondary endpoint, there were no significant difference in weight, systemic oxygen saturation, but in sex, age at BPV, and Z score of the PVD. Early palliative BPV prevented early surgical intervention in half of the neonates and young infants with TOF, which depended upon the degree of infundibular obstruction. However, early palliative BPV did not contribute to avoid transanular patch right-ventricular outflow repair among them.

Decreased Pulmonary Arterial Compliance is a Predictor for Poor Outcomes in Infants with Isolated Atrial Septal Defect and Pulmonary Hypertension.

This study aimed to investigate risk factors for poor outcomes in infants with isolated atrial septal defect (ASD) and pulmonary hypertension who had unexpectedly fatal course. We retrospectively reviewed 22 infants with isolated ASD and pulmonary hypertension, and analyzed the relationship between clinical outcomes and pulmonary hemodynamic parameters including pulmonary arterial resistance (Rp) and compliance (Cp) based on cardiac catheterization among them. Age and weight at cardiac catheterization were 5 (1-11) months and 4.9 (3.1-9.2) kg, respectively. There were 17 individuals with Down syndrome. Pulmonary hemodynamic parameters were shown as follows: the ratio of pulmonary to systemic blood flow: 2.0 (0.6-3.8), mean pulmonary arterial pressure: 41 (20-60) mmHg, the ratio of pulmonary to systemic mean pressure (Pp/Ps): 0.67 (0.46-1.13), Rp: 4.11 (0.68-15.80) Wood units m2, and Cp: 1.80 (0.63-6.16) mL/mmHg m2. There were 4 deaths during the follow-up period of 40 (7-241) months. Univariable logistic regression analysis revealed that Pp/Ps (odds ratio [OR]: 18,500, 95% confidential interval [CI] 1.48-230,659,690, P = 0.041) and Cp (OR: 0.03, 95% CI 0.001-0.73, P = 0.031) were significantly related to death. A Pp/Ps cutoff value more than 0.94 and a Cp cutoff value less than 0.97 mL/mmHg m2 yielded as the predictors of death with sensitivity of 98% and 94%, specificity of 75% and 75%, respectively. Decreased Cp is a previously unrecognized predictor for poor outcome in infants with isolated ASD and pulmonary hypertension.

Inducible torsades de pointes during an acute face immersion test in an adolescent with type 2 long QT syndrome.

We present the case of a 12-year-old boy with type 2 long QT syndrome in whom torsades de pointes was induced by an acute face immersion test. This test is feasible to predict cardiac events in adolescents with long QT syndrome.

Sub-clinical myocardial ischaemia due to cardiac strangulation by an epicardial pacemaker lead.

We present the case of a 10-year-old boy with congenital complete atrioventricular block who had cardiac strangulation by an epicardial pacemaker lead placed during infancy. Coronary angiography and Tc99m tetrofosmin myocardial perfusion scintigraphy suggested sub-clinical myocardial ischaemia.

Peripartum Management of Pregnant Women With Congenital Heart Disease.

BACKGROUND: The average maternal age at delivery, and thus the associated maternal risk are increasing including in women with congenital heart disease (CHD). A comprehensive management approach is therefore required for pregnant women with CHD. The present study aimed to investigate the factors determining peripartum safety in women with CHD.Methods and Results:We retrospectively collected multicenter data for 217 pregnant women with CHD (age at delivery: 31.4±5.6 years; NYHA classifications I and II: 88.9% and 7.4%, respectively). CHD severity was classified according to the American College of Cardiology/American Heart Association guidelines as simple (n=116), moderate complexity (n=69), or great complexity (n=32). Cardiovascular (CV) events (heart failure: n=24, arrhythmia: n=9) occurred in 30 women during the peripartum period. Moderate or great complexity CHD was associated with more CV events during gestation than simple CHD. CV events occurred earlier in women with moderate or great complexity compared with simple CHD. Number of deliveries (multiparity), NYHA functional class, and severity of CHD were predictors of CV events. CONCLUSIONS: This study identified not only the severity of CHD according to the ACC/AHA and NYHA classifications, but also the number of deliveries, as important predictive factors of CV events in women with CHD. This information should be made available to women with CHD and medical personnel to promote safe deliveries.

Pulmonary arterial compliance is a useful predictor of pulmonary vascular disease in congenital heart disease.

Histopathological assessment of the pulmonary arteries is crucial to determine the surgical indications in patients with congenital heart disease (CHD) and intractable pulmonary vascular disease (PVD). We aimed to clarify whether pulmonary hemodynamic parameters can predict PVD in patients with CHD and pulmonary arterial hypertension (PAH) We performed histopathological evaluations of lung specimens and cardiac catheterizations in 27 patients with CHD-PAH. We divided these patients into the patients with and without PVD, and compared pulmonary hemodynamic parameters including pulmonary arterial compliance (Cp) between two groups. Age at lung biopsy was 4 (2-7) months. There were 16 patients with trisomy 21. Cardiac diagnosis included ventricular septal defect in 16, atrial septal defect in 5, atrioventricular septal defect in 4, and others in 2. There were 11 patients with histopathologically proven PVD (Heath-Edwards classification grade ≥ 3 in 5; the index of PVD ≥ 1.1 in 3; extremely thickened media in 6; hypoplasia of the pulmonary arteries in 3). Cp in the patients with PVD was significantly lower than that in patients without PVD (0.99 [0.74-1.42] vs 1.56 [1.45-1.88], p = 0.0047), although there was no significant difference in the ratio of systemic to pulmonary blood flow, pulmonary arterial pressure, and resistance between two groups. A Cp cutoff value of < 1.22 ml/mmHg m2 as a predictor of PVD yielded a sensitivity and a specificity of 93% and 64%, respectively. Pulmonary arterial compliance can be a predictor of PVD among patients with CHD-PAH.