Corrigendum: Use of the index of pulmonary vascular disease for predicting longterm outcome of pulmonary arterial hypertension associated with congenital heart disease.

[This corrects the article DOI: 10.3389/fcvm.2023.1212882.].

Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease.

Aims: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH. Methods: This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death. Results: The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD <2.0 (P = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45-13.73; P = .009). Conclusions: The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered.

[Stanford Type A Acute Aortic Dissection with Myonephropathic Mrtabolic Syndrome].

A 57-year-old man was transferred with sudden onset chest pain and evolving paralysis and numbness in the left leg. Contrast computed tomography (CT) revealed Stanford type A acute aortic dissection from the ascending aorta to bilateral internal and external iliac arteries with blood flow obstruction to the left kidney and left lower limb. Surgery was initiated 10 hours after onset of ischemic symptoms in the leg. Femoro-femoral bypass was carried out first, and we ensured sufficient phlebotomy from the ischemic limb during reperfusion and continuous hemodiafiltration to prevent myonephropathic metabolic syndrome. Total aortic arch replacement was then performed. Our treatment strategy was effective in this case of Stanford type A aortic dissection with prolonged lower limb ischemia. Although left hip disarticulation was subsequently required due to intractable infection, the patient became able to walk with an artificial limb after post-rehabilitation.

Surgical repair of the adult form of scimitar syndrome.

Scimitar syndrome has various clinical presentations and anatomic features, and some cases are diagnosed in adulthood. Reconstruction using autologous tissue to reroute the right pulmonary venous blood flow to the left atrium is ideal. However, if the scimitar vein drains to the caudal segment of the inferior vena cava, reconstruction using prosthetic material may be necessary due to the distance between the left atrium and the scimitar vein. We describe the case of a 16-year-old boy with scimitar syndrome. We anastomosed the scimitar vein to the right atrium using an artificial graft and created an atrial septal defect for rerouting the right pulmonary venous blood to the left atrium. It has been 9 years since this procedure, and the patient has not experienced graft stenosis or thromboembolic events.

Being Overweight Is Related to Neoaortic Sinus Dilatation After Arterial Switch Operation.

BACKGROUND: Neoaortic sinus dilatation is a late comorbidity after an arterial switch operation in patients with dextro-transposition of the great arteries. We aim to explore whether neoaortic sinus dilatation is related to overweight or obesity, as these affect aortic remodeling in such patients. METHODS: We measured neoaortic diameters including those for the annulus, sinus, sinotubular junction, and ascending aorta by echocardiography and studied the relationship between these diameters and body mass index in patients aged 15 years and older after an arterial switch operation. RESULTS: A total of 45 patients were studied. Median (interquartile range) age, body mass index, and systolic blood pressure at echocardiography were 20 (17-28) years, 21.2 (18.4-24.2) kg/m2, and 120 (112-127) mm Hg, respectively. Echocardiogram revealed the following median values (interquartile ranges): neoaortic annulus diameter, 22.2 (19.8-23.8) mm; sinus diameter, 34.6 (31.8-39.8) mm; sinotubular junction diameter, 27.5 (22.4-30.0) mm; and ascending aortic diameter, 20.4 (19.4-22.7) mm. The neoaortic sinus diameter was significantly correlated with body mass index (r = 0.41, P = .004) and was significantly larger in patients with concomitant ventricular septal defect (P < .001) and those who were over-weight (body mass index ≥25 kg/m2; P = .006). CONCLUSION: Neoaortic sinus dilatation after an arterial switch operation is associated with being overweight during adolescence and young adulthood. It is important to prevent obesity in patients after an arterial switch operation and educate them on a healthy lifestyle.

Fetal cardiac capillary hemangioma resulting in tachyarrhythmia.

We present the case of a fetus with cardiac capillary hemangioma in the right atrial cavity. The tumor showed dramatic growth between the 28th and 32nd week of gestation and resulted in tachyarrhythmia. The patient was born at the 33 weeks of gestation weighing 2430 g via urgent cesarean section because the rapidly growing cardiac tumor caused incessant tachyarrhythmia, pericardial effusion, and fetal circulatory incompetence. Coronary angiography revealed that the right coronary artery drained into the tumor. Due to hemodynamic deterioration, the patient underwent subtotal resection of the tumor on the 2nd day after birth. Histopathological examination revealed an undifferentiated capillary hemangioma. The patient was discharged at the age of 86 days, as the tachyarrhythmia and hemodynamic incompetence had subsided; however, bradycardia and intermittent atrioventricular conduction disturbance gradually developed. Capillary hemangioma, a rare primary cardiac space-occupying tumor in children, can invade the conduction system.

Late Adverse Events in Patients With Pulmonary Atresia With Intact Ventricular Septum After Valvuloplasty.

BACKGROUND: We aimed to explore the relation between temporal right heart growth and long-term outcomes in patients with pulmonary atresia with an intact ventricular septum (PA/IVS) who underwent balloon pulmonary valvuloplasty (BPV). METHODS: We performed echocardiography to measure pulmonary valve diameter, right atrial end-systolic area (RAA), and right ventricular end-diastolic area (RVA) before BPV 1 and 5 years after BPV. Primary and secondary end points were to explore temporal changes in right heart structures and to determine echocardiographic parameters related to late adverse events (LAEs). RESULTS: In 31 patients, pulmonary valve diameter significantly increased after BPV, whereas tricuspid valve diameter remained unchanged throughout the 10.0-year follow-up (range, 5.8-14.0 years). After BPV, RAA temporally decreased, whereas RVA significantly increased. There were LAEs in 6 patients (19%), arrhythmias in 2, heart failure in 1, reintervention of the right ventricular outflow tract in 1, and reintervention for residual cyanosis in 2. The rate of freedom from LAEs at 5 and 10 years was 92% and 82%, respectively. Right atrial end-systolic area temporally decreased in patients without LAEs (P < .01); however, RAA remained unchanged throughout the period in patients with LAEs (P = .16). Moderate or severe pulmonary regurgitation (hazard ratio = 23.0; 95% confidence interval, 1.3-385; P = .03) and the ratio of RVA to RAA at 1 year after BPV (hazard ratio = 6.3 × 10-11; 95% confidence interval, 2.1 × 10-20 to 0.19; P = .03) were independent risk factors for LAEs. CONCLUSIONS: Disproportional right heart growth was observed in patients with pulmonary atresia with an intact ventricular septum after BPV. Pulmonary regurgitation and increased RAA are crucial for identifying the burden of LAEs among them.

Postoperative Maximal Aortic Diameter is a Significant Predictor of Dilation of the Residual Dissected Aorta after Aortic Replacement for Acute Debakey Type I Aortic Dissection.

BACKGROUND: This study investigated the impact of aortic diameter on late aortic dilation of the residual dissected aorta after tear-oriented aortic replacement for acute DeBakey type I aortic dissection. METHODS: Of 133 patients who underwent aortic replacement for acute DeBakey type I/II aortic dissection between 2008 and 2019, 45 patients with a residual dissected aorta after surgery for acute DeBakey type I aortic dissection and who underwent computed tomography at predischarge and after 1 year were retrospectively assessed. The aortic diameter and false lumen area were measured at 3 levels: the maximal aortic site, seventh thoracic vertebra, and celiac axis. Multivariable Cox regression analysis was employed to identify the predictors of late aortic dilation, defined as an aortic growth rate of ≥5 mm/year or a maximal aortic diameter of ≥55 mm. RESULTS: During a median follow-up of 75 [range: 13-152] months, 6 patients (5 men; mean age: 57 ± 14 years) experienced aortic dilation. All 6 patients had the maximal aortic diameter between the distal aortic arch and seventh thoracic vertebra level at the last computed tomography. Multivariable Cox regression analysis showed that the predischarge maximal aortic diameter was an independent determinant of late aortic dilation (hazard ratio: 2.28/mm, 95% confidence interval: 1.10-5.86). CONCLUSIONS: Predischarge maximal aortic diameter is a significant predictor of late aortic dilation in patients with a residual dissected aorta after tear-oriented surgical repair of acute DeBakey type I aortic dissection.

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.

Coronary artery patch augmentation for congenital left coronary ostial stenosis in Williams syndrome.

Left coronary ostial stenosis, which is associated with sudden death, occasionally occurs in individuals with Williams syndrome. However, surgical methods that provide reliable long-term revascularization remain unknown among infants and young children with coronary ostial stenosis. We describe the case of an 18-month-old boy with Williams syndrome who presented with cardiogenic shock due to left coronary ostial stenosis. We performed patch augmentation of the left coronary ostium using glutaraldehyde-treated autologous pericardium. At the last follow-up, the patient was well without any adverse events or myocardial ischemia.

Total anomalous pulmonary venous connection mimicking hypoplastic left heart syndrome.

A newborn with supracardiac total anomalous pulmonary venous connection vein presented the small left ventricle with z score of -7.5, retrograde blood supply in the transverse arch, and the dutcus-dependent systemic circulation. The patient underwent the repair of the anomalous pulmonary vein and bilateral pulmonary arterial banding soon after the birth and then transcatheter pulmonary arterial debanding at the age of 10 months because of an appropriate growth of the left ventricle.

Unilateral Embolization of the Internal Iliac Artery for Endovascular Aortic Repair Does Not Induce Gluteal Muscle Atrophy.

BACKGROUND: To investigate the effect of unilateral internal iliac artery (IIA) embolization for endovascular aortic repair (EVAR) on gluteal muscle size. METHODS: We assessed the gluteal muscle size in 111 consecutive patients who underwent elective EVAR with unilateral IIA embolization (n = 31) or without IIA embolization (n = 80) for abdominal aortic and/or iliac artery aneurysm. The cross-sectional area (CSA) of the gluteus maximus (Gmax) and gluteus medius/minimus (Gmed/min) was measured on computed tomography preoperatively, 6 months postoperatively, and final follow-up. Mean changes in the Gmax and Gmed/min CSA were evaluated using a mixed model analysis of variance. RESULTS: In the patients with embolization, both the Gmax and Gmed/min CSA significantly decreased over time on the embolization and nonembolization sides (P < 0.001); however, embolization did not affect the changes in the Gmax CSA (P = 0.64) and Gmed/min CSA (P = 0.99). In the patients with embolization and those without embolization, both the Gmax and Gmed/min CSA significantly decreased over time (P < 0.001); however, embolization did not affect the changes in the Gmax CSA (P = 0.76) and Gmed/min CSA (P = 0.11). CONCLUSIONS: Unilateral IIA embolization was not associated with gluteal muscle atrophy after EVAR. Pre-emptive unilateral IIA embolization for EVAR seems to be an acceptable procedure in terms of maintenance of gluteal muscle size.

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.

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.

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.

A case of cardiac strangulation following epicardial pacemaker implantation.

An 8-year-old boy had undergone permanent epicardial pacemaker implantation with a Y-shaped bipolar ventricular lead on day 6 after birth for treatment of congenital complete atrioventricular block. He was found to have pulmonary stenosis and mitral stenosis by follow-up echocardiography. Further studies including computed tomography and cardiac catheterization revealed that the pacemaker lead had completely encircled the cardiac silhouette and was in a state of "cardiac strangulation". We removed the previous pacing leads and generator and implanted a new epicardial dual-chamber pacing system in the right atrium and right ventricle. Additionally, an expanded polytetrafluoroethylene sheet was placed between the new leads and the heart to prevent recurrence of cardiac strangulation.

Atypical Hemolytic Uremic Syndrome following Acute Type A Aortic Dissection.

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA)-related disease that manifests as a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI) and is caused by uncontrolled activation of the complement system. We report the case of a 61-year-old woman with acute type A aortic dissection that subsequently developed into aHUS. The hematologic disorders underlying aHUS improved after treatment with the complement inhibitor eculizumab. It is important to consider aHUS when a patient clinically develops a triad of microangiopathic hemolytic anemia, thrombocytopenia, and an increasing creatinine level following cardiovascular surgery.

Impacts of surgical interventions on the long-term outcomes in individuals with trisomy 18.

OBJECTIVE: We aim to clarify whether surgical interventions can contribute to improve the long-term outcomes among individuals with trisomy 18. METHODS: We retrospectively studied 69 individuals with trisomy 18 admitted to 4 tertiary neonatal centers between 2003 and 2017. A cohort was divided into two groups: subjects with surgical interventions and conservative treatments. We compared the rates of survival and achieving homecare between the groups. RESULTS: Gestational age and birth weight were 37 (27-43) weeks and 1,700 (822-2,546) g, respectively. There were 68 patients with congenital heart disease and 20 patients with digestive disease. Surgical interventions including cardiac and digestive surgery were provided in 41% of individuals. There was no difference in gestational age (p=0.30), birth weight (p=0.07), gender (p=0.30), and fetal diagnosis (p=0.87) between the groups. During the median follow up duration of 51 (2-178) months, overall survival rates in 6, 12 and 60 months were 57%, 43% and 12%, respectively. Survival to hospital discharge occurred in 23 patients, and the rates of achieving homecare in 1, 6, and 12 months are 1%, 18% and 30%, respectively. There was no significant difference in survival rate (p=0.26) but in the rate of achieving home care (p=0.02) between the groups. Cox hazard analysis revealed that prenatal diagnosis (hazard ratio 0.30, 95%CI: 0.13-0.75), cardiac surgery (hazard ratio 2.40, 95%CI:,1.03-5.55), and digestive surgery (hazard ratio 1.20, 95%CI: 1.25-3.90) were related to the rate of achieving homecare. CONCLUSION: Aggressive surgical interventions contribute not to the long-term survival but to achieve homecare among individuals with trisomy 18. EVIDENCE LEVEL: Level 3 (Prognostic study, Case-Control study).

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.

Use of computed tomography-guided biopsy to detect Peptostreptococcus micros-induced mycotic abdominal aortic aneurysm after endovascular repair.

Mycotic aortic aneurysm (MAA) is rare but lethal. Detection of the causative bacteria is important for successful treatment. In some cases, however, no micro-organisms are detected by repeated blood cultures. Open surgery is the standard procedure for MAA, but endovascular intervention is also performed. An aneurysm specimen cannot be taken for culture when using an endovascular approach, decreasing the opportunity to detect the causative organism. We performed successful endovascular repair in a rare case of a Peptostreptococcus micros-induced MAA as detected by postoperative computed tomography-guided biopsy. This case may help to establish appropriate medical treatment for MAAs.