Clinical Prognosis of Vocal Cord Paralysis After Cardiothoracic Surgery in Infants.

We aimed to clarify the long-term outcomes and prognosis of vocal cord paralysis (VCP) after cardiothoracic surgery in infants as well as the usefulness of laryngeal ultrasound (LUS) as screening for VCP. Overall, 967 infants aged 1-year-old or younger who underwent cardiothoracic surgery between 2008 and 2022 were included in this study. We divided the patients into two groups based on the period on whether they underwent screening without or with LUS and compared the incidence of VCP between the groups. There were no differences in the patients' preoperative characteristics between the two periods, whereas the incidence of VCP was significantly higher in period 2 than in period 1 (11.0% vs. 3.2%, p < 0.0001). The incidence of VCP among the procedures, including aortic arch repair, was > 50% and significantly increased from period 1 to period 2. The sensitivity and specificity of LUS was 87% and 90%, respectively. Symptoms of VCP improved in 92% of patients. Repeated flexible laryngoscopy revealed that the residual rate of VCP was 68%, 52%, and 48% at 6, 12, and 24 months, respectively. In conclusion, symptoms of postoperative VCP improved in most cases; however, paralysis persisted in half of the patients. As a screening method, LUS is useful for evaluating postoperative VCP. A more accurate understanding of VCP is needed to improve postoperative outcomes.

Congenital Pulmonary Lymphangiectasia Masked by Postoperative Pulmonary Venous Obstruction in an Infant with Total Anomalous Pulmonary Venous Connection.

Congenital pulmonary lymphangiectasia (CPL) is associated with fetal pulmonary venous obstructive physiology. The precise morbidity of CPL is unknown as CPL is generally fatal in neonates. Here, we report an infant with secondary CPL in total anomalous pulmonary venous connection (TAPVC). He developed severe pulmonary hypertension (PH) after corrective surgery for TAPVC. However, cardiac catheterization showed mild left pulmonary venous obstruction (PVO), which was deemed unnecessary for re-intervention. He died at 11 months-old due to an exacerbation of PH. Autopsy revealed medial hypertrophy of the pulmonary arteries, mild left PVO, and marked dilatation and proliferation of the pulmonary lymphatics which might have been involved in the PH, although CPL was not conclusively identified based on the previous biopsy findings. We should be aware of the possibility of CPL in addition to postoperative PVO when encountering patients with fetal pulmonary venous obstructive physiology. Furthermore, a cautious approach to the interpretation of lung biopsy results is warranted.

Efficacy of Short-Term Oral Prednisolone Treatment in the Management of Pericardial Effusion Following Pediatric Cardiac Surgery.

A standard treatment for pericardial effusion without cardiac tamponade after pediatric cardiac surgery has not been established. We evaluated the efficacy of short-term oral prednisolone administration, which is the initial treatment for postoperative pericardial effusion without cardiac tamponade at our institution. Between October 2008 and March 2020, 1429 pediatric cardiac surgeries were performed at our institution. 91 patients required postoperative treatment for pericardial effusion. 81 were treated with short-term oral prednisolone. Pericardial effusion was evaluated using serial echocardiography during diastole. Pericardial drainage was performed for patients with circumferential pericardial effusion with a maximum diameter of ≥ 10 mm or signs of cardiac tamponade. Short-term oral prednisolone treatment was administered to patients with circumferential pericardial effusion with a maximum diameter of < 10 mm or localized pericardial effusion with a maximum diameter of ≥ 5 mm. Patients with localized pericardial effusion with a maximum diameter of < 5 mm were observed. Prednisolone (2 mg/kg/day) was administered orally for 3 days, added as needed. Short-term oral prednisolone treatment was effective in 71 cases and 90% of patients were regarded as responders. The remaining patients were deemed non-responders who required pericardial drainage. Overall, 55 responders were deemed early responders whose pericardial effusion disappeared within 3 days. There were no cases of deaths, infections, or recurrence of pericardial effusion. The amount of drainage fluid on the day of surgery was higher in the non-responders. In conclusion, short-term oral prednisolone treatment is effective and safe for treating pericardial effusion without cardiac tamponade after pediatric cardiac surgery.

Insufficient development of vessels and alveoli in lungs of infants with trisomy 18-Features of pulmonary histopathological findings from lung biopsy.

The aim of this study was to evaluate the features of pulmonary histopathological changes in cases of trisomy 18 complicated with congenital heart disease and pulmonary arterial hypertension. Twenty-eight patients with trisomy 18 underwent open lung biopsy at the time of primary operation in our hospital between 2008 and 2019. We compared these histopathological findings with those from previously described groups without trisomy 18. Mean age at primary cardiac surgery was 37 days (range, 9-69 days). According to the Heath-Edwards (HE) classification, 1, 8, 12, and 5 patients were graded as 0, 1, 2, and 3, respectively, whereas 2 patients were not classifiable due to medial defects in the small pulmonary arteries (MD). Four (14.3%) and 13 (46.4%) patients presented with MD and hypoplasia of the small pulmonary arteries (HS). Fifteen (53.6%) and 21 (75.0%) patients presented with alveolar hypoplasia (AH) and alveolar wall thickening (AT). MD, HS, and AH in trisomy 18 were present frequently, differing significantly from previous reports. These findings might be associated with congenital inadequate development of vessels and alveoli in the lung, contributing to a high risk of PAH in trisomy 18.

Pulmonary vasodilators can lead to various complications in pulmonary "arterial" hypertension associated with congenital heart disease.

Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) is one of the major complications in patients with CHD. A timely closure of the left-to-right shunt will generally result in the normalization of the pulmonary hemodynamics, but a few patients have severe prognosis in their early childhood. We hypothesized that wide-ranging pathological mechanism in PAH could elucidate the clinical state of severe CHD-PAH. Using electronic medical records, we retrospectively analyzed six infants with severe CHD-PAH who had treatment-resistant PH. All patients were born with congenital malformation syndrome. After starting on a pulmonary vasodilator, five of the six patients developed complications including pulmonary edema and interstitial lung disease (ILD), and four patients had alveolar hemorrhage. After steroid therapy, the clinical condition improved in four patients, but two patients died. The autopsy findings in one of the deceased patients indicated the presence of recurrent alveolar hemorrhage, pulmonary venous hypertension, ILD, and PAH. Based on the clinical course of these CHD-PAH in patients and the literature, CHD-PAH can occur with pulmonary vascular obstructive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH), ILD, and/or alveolar hemorrhage. The severity of CHD-PAH may depend on a genetic disorder, respiratory infection, and upper airway stenosis. Additionally, pulmonary vasodilators may be involved in the development of PVOD/PCH and ILD. When patients with CHD-PAH show unexpected deterioration, clinicians should consider complications associated with PVOD/PCH and/or pulmonary disease. In addition, the choice of upfront combination therapy for pediatric patients with CHD-PAH should be selected carefully.

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.

Prophylactic Opening of the Pleural Cavity for Postoperative Drainage is a Risk Factor for Prolonged Pleural Effusion After a Fontan Operation.

Postoperative prolonged pleural effusion (PPE) remains a confounding problem after a Fontan operation. We aimed to describe the risk factors for PPE after a Fontan operation and to clarify the impact of prophylactic opening of the pleural cavity (POPC) for drainage tube insertion on PPE. We retrospectively reviewed the medical charts of 50 consecutive patients who underwent a Fontan operation at our institution. POPC for postoperative drainage was performed based on each surgeon's preference. Patients were divided into three groups for analysis: group A (n = 12), no opening; group B (n = 14), unilateral opening; and group C (n = 24), bilateral opening. At the time of surgery, the median age of our patient group was 26 months, with a median body weight of 10.5 kg. The volume of pleural effusion tended to be lower in group A than in groups B and C (p = 0.08). The median duration of drainage was significantly shorter (p = 0.03) in group A (3 days) than in group B (4 days) or C (5 days). Overall, 12 patients required chest tube drainage for ≥ 7 days. Multivariate analysis revealed POPC (p = 0.01) and postoperative water balance (p = 0.03) as independent predictors of PPE. POPC and postoperative water balance are risk factors for PPE after a Fontan operation. Therefore, avoiding POPC for postoperative drainage may reduce the risk of postoperative pleural effusion and morbidities associated with PPE after a Fontan operation.

Is Trisomy 21 a Risk Factor for Rapid Progression of Pulmonary Arteriopathy?　- Revisiting Histopathological Characteristics Using 282 Lung Biopsy Specimens.

BACKGROUND: Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.Methods and Results:A retrospective review of a lung biopsy registry identified 282 patients: 188 patients with trisomy 21 (Group D) and 94 without (Group N). The mean age at lung biopsy was 3 and 7 months (P<0.0001). Pulmonary arterial pressure (PAP) and pulmonary vascular resistance were similar between the 2 groups. There were no significant differences in the proportion of patients with irreversible intimal lesions or the index of pulmonary vascular disease (IPVD; a measure of the degree of pulmonary arteriopathy progression) between the 2 groups. In addition, after propensity score matching for patient background (n=43 in each group), there were no significant differences in IPVD (P=0.29) or the ratio of irreversible intimal changes between the D and N groups (P=0.39). Multivariate analysis identified age (P<0.0001) and PAP (P=0.03) as the only risk factors for progression of pulmonary arteriopathy. CONCLUSIONS: Histopathologically, early progression of pulmonary arteriopathy in patients with trisomy 21 was not proved compared with patients without trisomy 21. Although we cannot exclude the possibility of bias in the Group D and N patients who were slated for lung biopsy, factors other than pulmonary arteriopathy may affect the marked progression of clinical PH in trisomy 21 patients.

Evidence of Pulmonary Vascular Reverse Remodeling After Pulmonary Artery Banding Performed in Early Infancy in Patients With Congenital Heart Defects.

BACKGROUND: Histomorphometric evidence of the effect of pulmonary artery banding (PAB) in infancy on pulmonary vascular reverse remodeling has not been fully described.Methods and Results:We retrospectively reviewed 34 patients who underwent serial lung biopsies before and after PAB.Index of pulmonary vascular disease (IPVD) as a measure of the degree of progression of pulmonary arteriopathy significantly decreased after PAB (1.22±0.25 at 1st and 1.13±0.21 at 2nd biopsy, P=0.04). Additionally,DR=100 µmas an indicator of medial thickness of pulmonary arteries significantly decreased after PAB (15.6±3.7 at 1st and 11.4±2.6 at 2nd biopsy, P<0.0001). Patients were divided into 3 groups by age at PAB: <3 months (Group 1), between 3 and 6 months (Group 2), and >6 months (Group 3). The average secondDR=100 µmof groups 1 and 2 was significantly lower than that of group 3 (11.1±2.2 and 9.8±2.0 vs. 14.9±2.8, respectively; P<0.0001). Additionally, the second IPVD was also significantly lower in groups 1 and 2 than in group 3 (1.1±0.2 and 1.1±0.2 vs. 1.3±0.4, respectively; P=0.02). CONCLUSIONS: Histomorphometric evidence of post-PAB pulmonary vascular reverse remodeling is robust. The magnitude of vascular reversibility is pronounced when PAB is performed before 6 months of age.

Predictors of the need for pacemaker implantation after the Cox maze IV procedure for atrial fibrillation.

PURPOSE: The Cox maze IV (CMIV) procedure is being used increasingly frequently for surgical ablation of atrial fibrillation (AF). This study aimed to identify the risk factors of the need for postoperative pacemaker implantation (PMI) after CMIV. METHODS: Preoperative, intraoperative, and postoperative data were retrospectively collected from 67 consecutive patients who underwent CMIV at our institution; 7 (10.4%) required PMI (as a treatment of brady AF or sick sinus syndrome). RESULTS: Patients who needed PMI tended to have lower preoperative heart rates than those who did not on a 12-lead electrocardiogram (ECG; 68.7 ± 11.6 vs. 79.1 ± 18.5 bpm, p = 0.07) and a 24-h ECG (94,772 ± 9800 vs. 109,854 ± 19,078 beats/day, p = 0.03). A multivariate analysis identified a low amplitude of the fibrillatory wave on preoperative ECG as a risk factor of PMI necessity after CMIV [odds ratio = 14.7; 95% confidence interval (CI) 1.9-324.7; p = 0.007] and internal use of angiotensin-converting enzyme inhibitors or angiotensin receptor blockers (ACEIs/ARBs) as a negative risk factor (odds ratio = 0.16; 95% CI 0.02-0.99; p = 0.049). CONCLUSIONS: A low amplitude of the fibrillatory wave was identified as a risk factor of PMI necessity, whereas the internal use of ACEIs/ARBs diminished the need for PMI. These factors should be considered before CMIV is performed.

Adventitial Cystic Disease Communicating with the Knee Joint: A Case Report with Histopathological Study of the Connection.

Adventitial cystic disease is a rare nonatheromatous cause of popliteal artery disease. Here, we present a case of a 51-year-old male patient who presented with right calf claudication caused by adventitial cystic disease. Preoperative magnetic resonance imaging and intraoperative findings revealed the presence of a connection between the cyst and adjacent knee joint. In addition, histopathological examination revealed that the tissue structure of the connection was similar to that of adventitial cysts. The tissue composed of 2 types of cells, namely macrophages and fibroblast-like cells, and lesional cells expressed D2-40. These findings supported the ganglion theory as the underlying physiopathology of this disease and were helpful in deciding the management of this case.

[Issues in Coronary Artery Bypass Grafting for Kawasaki Disease].

Progressive narrowing at the entrance and exit of coronary artery aneurysm can develop at late phase of Kawasaki disease (KD). Evaluation and prediction of progressive coronary lesions remain a challenge in the treatment of post-KD coronary artery disease. We aimed to elucidate long-standing issues imposed on the patients who underwent coronary artry bypass grafting (CABG) for coronary artery lesions associated with KD. Between January, 2000 and December, 2013, CABG for coronary artery lesions associated with KD were performed in 6 patients (male/female: 5/1, mean age 14.5±10.0). There was no operative mortality. Follow-up for the 6 patients has been performed with the average period of 5 years (1~9 years). Cardiac events occurred in 2 patients during follow-up. In 1 patient, left internal thoracic artery (LITA) occluded due to flow competition between the native artery and LITA graft after LITA to LAD bypass grafting. The other patient required a re-do CABG using the free right internal thoracic artery to the circumflex branch because of occlusion at the coronary artery aneurysms after 4 years postoperatively. Meticulous preoperative diagnostic evaluations of coronary artery aneurysm may further improve the long-term outcome after surgical intervention for coronary lesions in conjunction with an aneurysm.

[Mycotic Aneurysm of Distal Aortic Arch, after Total Arch Replacement with Open-stent Graft;Report of a Case].

The patient was a 72-year-old man, who had undergone total arch replacement with an open-stent graft due to saccular aneurysm of distal arch, 2 years before. He was admitted to a local hospital with the complaint of high fever, and was diagnosed as having pyothorax, after computed tomography (CT) scanning. After transferred to our hospital, he was treated by drainage, and antibiotic therapy. But CT scans showed the enlargement of distal arch aneurysm, and migration of the stent graft. Urgent operation was performed. We approached to the site by a full sternotomy, and left anterolateral thoracotomy. Segment 1+2 of the left lung was resected to avoid bleeding and lung injury. Graft replacement of distal arch and descending aorta was performed on cardiopulmonary bypass, with hypothermia, selective brain perfusion and systemic circulatory arrest. To protect from recurrence of infection, the omental flap was transposed to the graft site. Until now, there is no recurrence of infection.

[Two cases of fulminant myocarditis with mesenteric ischemia].

Fulminant myocarditis (FM) can lead to a refractory cardiogenic shock and multiple organ failure. Mechanical circulatory support (MCS) is not infrequency required to salvage patients in a profound shock. Advanced technology in MCS such as a temporary ventricular assist device can yield improved early outcome. However, incessant mesenteric ischemia remains a challenge to be resolved even with MCS. We here-in report 2 cases of FM associated with mesenteric ischemia that was extremely difficult to diagnose and treat. Therapeutic strategy for FM with mesenteric ischemia is discussed.

[Discrete type subaortic stenosis disclosed by hemolytic anemia after aortic and mitral valve replacement].

We report a case of discrete type subaortic stenosis disclosed by hemolytic anemia 7 years after aortic and mitral prosthetic valve replacement. A 53-year-old female complained of general fatigue, dyspnea, macrohematuria and hemolysis. She had undergone aortic valve replacement for non-coronary cusp perforation 15 years before, and mitral valve replacement and tricuspid annuloplasty 7 years before. Echocardiography showed mitral prosthetic valve regurgitation (III/IV degree) and symptomatic hemolysis might be caused by accelerated blood flow through the prosthetic valve. A mild aortic stenosis (peak flow verocity:3.73 m/s) was also pointed out. The redo double valve replacement was performed. Intraoperative findings showed discrete type subaortic stenosis due to extensive pannus formation, but that the previously implanted prosthetic valves were intact. The blood flow biased by the interference of the subaortic stenosis might have obstructed closure of the mitral prosthetic valve and caused mitral regurgitation. Postoperatively, hemolysis and mitral regurgitation were diminished, and aortic stenosis was improved.

[Successful management of aorto-esophageal fistula, using staged 3 times operation].

A 66-year-old man, presented with hematemesis and hemorrhagic shock, was transported to our institution. Computed tomographic examination suggested a pseudo-aneurysm formed by bleeding from aorto-esophageal fistula( AEF). We planned staged operations. At first, in an emergent operation, graft replacement of descending aorta, was performed under partial cardiopulmonary bypass, by left thoracotomy approach. AEF was 5 mm in diameter, and existed inside of normal-diameter and non-aneurysmal aortic intima. AEF orifice into pseudo-aneurysm was closed with aortic wall and was sutured tightly. Next day, gastrointestinal fiberscopy was performed, and penetrating ulcer was found at lower esophagus.To prevent infective complications, the 2nd operation, subtotal esophagectomy, cervical esophagostomy, gastrostomy and tube ileostomy were performed by right thoracotomy approach. Post- operative course was uneventful. Two months later, 3rd operation, cervical esophago-gastric anastomosis was performed, uneventfully. Two years and four months elapsed, and no evidences of infection and formation of pseudo-aneurysm were observed.

Combined procedure using a double flap as a surgical option for coarctation of the aorta with delayed diagnosis.

Simple coarctation of the aorta is repaired in an infant by direct end-to-end anastomosis of the aorta or subclavian flap aortoplasty. However, some cases are not detected until late childhood. For school-age patients, greater consideration must be given to risks such as postoperative limb ischemia and the potentially harmful effects of any artificial material on future growth. Here, we describe our technique for these patients, in whom the value of direct anastomosis is uncertain, to minimize the amount of synthetic graft material used while achieving successful anatomical repair.

How do we treat severely prolapsed true ectopia cordis?

Ectopia cordis is an extremely rare condition where the heart displaces outside the thoracic cavity. Treating this, especially when accompanied by congenital heart diseases and severe protrusion, is challenging. Here, we present a case of successful treatment involving intracardiac repair and delayed sternal closure using local skin flaps.

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.].

[Ventricular septal rupture and right ventricular free wall rupture after acute myocardial infarction].

Cardiac rupture is a catastrophic complication of acute myocardial infarction with highly mortality rate. Three types of rupture are ventricular free wall rupture( VFR), ventricular septal rupture( VSR), and papillary muscle rupture( PMR). A combination of any 2 types of rupture is called ventricular double rupture (VDR), and very rare. We report a case of VDR (VSR and VFR) after acute myocardial infarction. A 76-year-old female with heart failure was admitted to our hospital. Echocardiography showed an apical VSR and pericardial effusion. She was diagnosed with VDR and emergent operation was performed. During operation, the site of VFR was right ventricle, which was the same infarction area of VSR. VSR was closed by infarction exclusion technique, concurrently excluding the site of VFR. VFR was successfully repaired by mattress sutures. Post-operative course was good without heart failure, though residual shunt was remained. The patient survived and was discharged from our hospital.