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.

Surgical Management of Group A ß-Hemolytic Streptococcal Aortic Root Abscess.

This case report presents a pediatric patient with an aortic root abscess caused by group A ß-hemolytic Streptococcus. After aggressive debridement of infected tissue, the continuity between the left ventricular outflow tract and aorta was reconstructed with autologous pericardium, and the aortic valve was replaced with a mechanical prosthesis using the Konno procedure with concomitant mitral valve plasty. We believe that radical resection of the abscess is an effective way to eradicate such a life-threatening infection.

Infectious Aneurysms Caused by Streptococcus Pyogenes in Children.

We describe the detailed clinical course of rapidly enlarging infective aneurysms during the treatment of endocarditis and purulent pericarditis caused by Streptococcus pyogenes . We show that S. pyogenes aneurysms can enlarge rapidly within 1-2 days. Moreover, we highlight the benefit of transporting patients to a facility offering multidisciplinary treatment, even if vital signs stabilize to the point.

Coronary Artery Z Score is Associated with Postoperative Outcomes in Patients with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular anomaly that occurs in approximately 1 in 300,000 live births. This study aimed at identifying preoperative predictors of immediate postoperative outcomes. We conducted a retrospective, cross-sectional, single-center study and reviewed echocardiographic and hemodynamic data from all patients before and after surgical repair of ALCAPA at our center from January 2004 to February 2018. In all cases, the left coronary artery arose from the main pulmonary artery or a major branch. A total of 10 patients (age 1 month to 10 years, median 3 months) underwent ALCAPA surgical repair during the study period. No patients required a left ventricular assist device (LVAD) before surgery, but 4 patients (40%) received an LVAD after the surgery. The left ventricular ejection fraction (LVEF) improved in all patients following surgery. The utility of preoperative factors associated with pre- and post-procedure LVEF was investigated. LV dimension, as well as right coronary artery (RCA) and left coronary circumflex artery (LCX) Z scores were associated with a higher LVEF in the preoperative state. Patients with larger RCA, left ascending artery (LAD), and LCX Z scores also had a shorter duration of mechanical ventilation and ICU stay following surgery. Patients with a RCA Z score < 4 required implantation of an LVAD postoperatively. ALCAPA patients with larger RCA and LCX demonstrated a higher preoperative LVEF, while those with larger RCA, LAD, and LCX had superior postoperative hemodynamics and clinical outcomes.

Pulmonary haemodynamics in Fontan physiology after lobectomy in a patient with a single ventricle associated with pulmonary sequestration.

A 2-year-old girl with a functionally univentricular heart associated with a pulmonary sequestration underwent right lower lobectomy after which increased lung volume with low mean pulmonary artery pressure and pulmonary vascular resistance was documented. A cardiac catheterisation performed after a subsequent total cavopulmonary connection demonstrated favourable Fontan haemodynamics. Lobectomy may have induced compensatory lung growth, contributing to the maintenance of haemodynamics favourable for the long-term success of the Fontan procedure.

Right ventricular centripetal plication: an aggressive right ventricular exclusion technique.

In patients with a functional single ventricle such as neonatal Ebstein's anomaly or pulmonary atresia with intact ventricular septum, the right ventricle can compress the left ventricle and decrease its performance due to the volume or pressure overload of the right ventricle. We have performed right ventricular centripetal plication from the inside to exclude the right ventricle and to minimize the adverse effect on the left ventricle and the results have been satisfactory.

Optimal Surgical Management Using a Classic Blalock-Taussig Shunt for an Infected Pseudoaneurysm After a Modified Blalock-Taussig Shunt Procedure.

We present 2 cases of a 3-month-old girl and boy who were diagnosed with an infected pseudoaneurysm 2 months after undergoing left-sided modified Blalock-Taussig shunt (mBTS) operations for pulmonary atresia. Because the shunts in both cases were nearly obstructed, they underwent a 2-stage surgical approach: classic BTS operations through a right thoracotomy to establish sufficient pulmonary flow and infected graft removal through a median sternotomy after close observation of the state of the aneurysms. By utilizing autologous tissue from a different thoracic entry, both patients were successfully managed and recovered without any recurrence of infection.

Peripheral Veno-Arterial Extracorporeal Membrane Oxygenation as a Bridge to Decision for Pediatric Fulminant Myocarditis.

It is essential to establish an appropriate initial treatment strategy for pediatric fulminant myocarditis. We reviewed eight cases of pediatric fulminant myocarditis that required extracorporeal membrane oxygenation (ECMO) from 2012 to 2015. The median age was 8 years (range 3 months-13 years), and the median body surface area was 0.89 m(2) (range 0.35-1.34 m(2) ). Peripheral veno-arterial ECMO was initially applied, and we evaluated whether heart decompression was sufficient. If the pump flow was insufficient, central cannulation was performed via median sternotomy (central ECMO). The need for subsequent ventricular assist device (VAD) support was determined 72 h after ECMO initiation. Six patients were bridged to recovery using peripheral ECMO support only (for 3-11 days), whereas two required VAD support. One patient was switched to central ECMO before VAD implantation. Three patients died of multiorgan failure, even though cardiac function recovered in two of those patients. The duration from hospital arrival to ECMO initiation was shorter in the survival (3.3 ± 1.3 h; range 1.6-4.7 h) than in the nonsurvival group (32 ± 28 h; range 0.7-55 h). Peripheral ECMO can be useful as a bridge to decision for pediatric fulminant myocarditis, which is frequently followed by a successful bridge to recovery. It is important to determine whether ECMO support should be initiated before organ dysfunction advances to preserve organ function, which provides a better bridge to subsequent VAD therapy and heart transplant or recovery.

Modified Damus-Kaye-Stansel Procedure Subsequent to Pulmonary Artery Banding.

Both the Damus-Kaye-Stansel procedure and pulmonary artery banding provide an effective palliative technique in certain subsets of patients, but this combination of procedures is potentially hazardous in pulmonary valve geometry. This is a matter of vital concern, especially in patients whose pulmonary arteries have to work after operations as the major systemic output routes. In this report, we present a novel surgical modification that builds a systemic output route without causing valve deformities.

Successful arterial switch operation for transposition of the great arteries with intact ventricular septum and congenital diaphragmatic hernia: A case report.

We report performing a successful arterial switch operation on a full-term male infant with transposition of the great arteries and intact ventricular septum associated with a congenital diaphragmatic hernia. The patient developed severe persistent pulmonary hypertension after successful repair of a left congenital diaphragmatic hernia that restricted pulmonary blood flow into the hypoplastic left lung, and subsequent unilateral pulmonary congestion of the unaffected right lung occurred because of the hemodynamics generated by the transposition of the great arteries. Intravenous epoprostenol with nitric oxide inhalation and mechanical hypoventilation effectively controlled pulmonary vascular resistance before the arterial switch operation was performed. .

Nonmycotic false aneurysm of aortic cannulation site presenting 26 years postoperatively.

We report the case of a 64-year-old woman who presented with a false aneurysm in the ascending aorta where arterial cannulation was done in an operation 26 years earlier. The aneurysm was excised with the ascending aorta and successfully replaced with a prosthetic graft during deep hypothermic circulatory arrest and retrograde cerebral perfusion, accompanied with concomitant procedures of mitral valve replacement and maze procedure. When the aneurysm, 3.5 x 3.0 x 4.5 cm, was removed, it showed a remarkable sharp line of demarcation between the normal aorta. Microscopic examination of the specimen was consistent with the features of a pseudoaneurysm.

Predictors affecting durability of epicardial pacemaker leads in pediatric patients.

OBJECTIVES: Despite pacemaker therapy in children and adolescents favoring an initial epicardial approach, predictors of lead failure have not been well clarified. The aim of this study was to assess the long-term outcomes and to determine predictors affecting lead durability in pediatric pacing therapy. METHODS: We reviewed the outcomes of 109 consecutive pacing leads implanted in 55 patients (median age, 5.2 years; range, 31 days-15.8 years), including 38 atrial and 71 ventricular leads. They consisted of 58 (53%) fishhooks, 37 (34%) screw-in leads, and 14 (13%) steroid-eluting suture-on leads. Seventy (64%) were implanted in patients with structural heart disease. RESULTS: The leads were followed for a median of 6.4 years (range, 3 days-22.9 years). Lead failure occurred in 29 leads (27%; median of 8.4 years after implantation). Exit block or elevation of pacing threshold was the most common cause (n = 18), but failures did not directly cause patient death. The overall 1-, 5-, 10-, and 15-year lead survivals were 100%, 89.0%, 72.5%, and 55.5%, respectively. Multivariate Cox analysis revealed concurrent structural heart disease (relative risk, 2.85; 95% confidence interval, 1.27-6.42; P = .011) to be the only significant predictor of lead failure. CONCLUSIONS: Epicardial leads provide a reliable technique for managing rhythmic disturbance problems in the pediatric population. The only significant predictor of lead failure is the presence of structural heart disease.

One-and-a-half ventricular repair through the right lateral thoracotomy: an alternative to midline approach in a patient with previous mediastinitis.

We report a patient who successfully underwent a one-and-a-half ventricular repair (1.5 VR) through a right lateral thoracotomy. In the case of possible hazardous complications at the sternal reentry because of previous mediastinitis, this approach was thought to be an option in selected patients to complete a functional correction by means of 1.5 VR.

The case of an explanted 16-year-old mitral Carpentier-Edwards pericardial bioprosthesis.

We report the case of a mitral Carpentier-Edwards pericardial bioprosthesis that was explanted from a 43-year-old female patient because of structural valve deterioration 16 years following implantation. Upon removal, the prosthesis was found to be discolored and all leaflets were stiff and hard, showing extensive calcification, pannus overgrowth, leaflet hematoma, and multiple disruptions. One leaflet presented a wavy free margin due to commissural disruptions, leading to incomplete cusp coaptation. The accumulated physical symptoms of the patient were consistent with these findings.

Midterm results for endovascular stent grafts via median sternotomy for distal aortic arch aneurysm.

Dislocation or perigraft leakage is a major problem during the follow-up period for endovascular stent graft (EVG) implantation via median sternotomy for distal aortic arch aneurysm. Anchoring sutures have been applied for the prevention of these complications. Here, we evaluate the midterm results and the efficacy of anchoring sutures. There were 21 male and 2 female patients enrolled in the study with an average age of 70 years (59-83). Aneurysm types were 21 true aneurysms (13 fusiform, 8 saccular), 1 chronic dissection, and 1 penetrating aortic ulcer. Total arch replacement was performed in two patients and aortocoronary bypass grafting was combined in four patients. Anchoring sutures to fix the EVG via median sternotomy were applied for 11 recent patients. The average follow-up period was 44 (22-79) months. There were no operative mortalities, but one case of paraplegia, one of stroke, and two of temporary spinal cord dysfunction. Complications related to EVG via median sternotomy occurred in five patients who had not received anchoring sutures, but the 11 patients with anchoring sutures showed no EVG-related complications. A chronic type B dissection revealed impending rupture due to false lumen infection 1 year after operation and repeat surgery was performed. Stent dislocation occurred in four patients, two of whom required repeat surgery via left thoracotomy due to endoleakage 3 and 4 years after surgery. One died from aneurismal rupture 5 years after surgery. One required emergency graft replacement because of graft perforation 5 years after surgery. The proportion of patients free from EVG-related events at 5 years after surgery was 75%. Anchoring sutures (P = 0.0155) and a large aneurysm of more than 80 mm (P = 0.0190) were predictors of late EVG-related complications. There were five late deaths: two were from stroke, one from rupture, one from cancer, and one from multiple organ failure after repeat surgery. EVG shortens postoperative recovery with relatively lower mortality, but spinal cord injury is a complication occurring with a relatively high rate. Patients with large aneurysms are not good candidates for EVG implantation via median sternotomy Anchoring sutures to fix the EVG to the aortic wall should help prevent EVG-related events and improve the durability of EVG.

Measurement of cerebral-oxygenation status when commencing cardiopulmonary bypass in pediatric open-heart surgery.

OBJECTIVE: We hypothesize that there is a difference in the cerebral-oxygenation status between cyanotic and non-cyanotic congenital heart disease when commencing a crystalloid-primed cardiopulmonary bypass (CPB). We tested this hypothesis by using near-infrared spectroscopy (NIRS). METHODS: Group 1 consisted of ten patients with non-cyanotic congenital heart diseases, including atrial septal (n=4) and ventricular septal defects (n=6), while group 2 consisted of ten patients with cyanotic congenital heart diseases, including tetralogy of Fallot (n=7) and univentricular heart (n=3). Changes in cerebral-oxygenated, deoxygenated and total hemoglobin concentrations were measured by NIRS just before and every minute for the first 10 min after commencing CPB. Arterial blood analysis was performed at those same time times. RESULTS: NIRS showed a rapid fall and plateauing of cerebral-oxygenated, deoxygenated and total hemoglobin in group 1. However, although group 2 showed a rapid fall and plateauing of cerebral-oxygenated hemoglobin, a rapid fall and continuous gradual decrease in cerebral-deoxygenated and total hemoglobin were also seen. Cerebral-deoxygenated and total hemoglobin decreased more markedly in group 2 than in group 1 (P<0.001, 0.01, respectively). CONCLUSION: NIRS revealed that the cerebral-oxygenated hemoglobin could be maintained at a similar level at the beginning of CPB in both groups. However, it showed a different distribution of cerebral-deoxygenated and total hemoglobin between the groups. An inadequate cerebral-oxygenation status may occur in the early phase of CPB in patients with cyanotic congenital heart diseases.

[Off-pump atrial septectomy in a small neonate].

We report a surgical technique for off-pump atrial septectomy using a Rongeur forceps in the case of a newborn infant weighing less than 2,000 grams. The 6-day-old patient suffered from hypoplastic left heart syndrome with a restrictive interatrial communication. A purse-string suture was placed on the right atrium. A hand-made dull-tipped cannula was inserted into the right atrium through the suture to probe the interatrial communication. Monitoring the pressure at the tip of cannula, we could clearly determine when the cannula tip entered the left atrium. Then, a Rongeur forceps was inserted instead of the cannula, and used to bite into the interatrial septum. It was removed slowly together with the resected atrial specimen. In this manner, an atrial septectomy can be performed without cardiopulmonary bypass; this is far safer and easier than other procedures, such as the Blalock-Hanlon technique. This method can be performed in a small neonate as well as an older infant in whom balloon atrial septostomy is difficult.