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.

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.

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.].

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.

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.

Regression of oesophageal varices in total anomalous pulmonary venous connection.

The coexisting of oesophageal varices with total anomalous pulmonary venous connection is extremely rare but contains a potential leading to a lethal haemorrhage. The fate of the oesophageal varices after total anomalous pulmonary vein connection repair remains largely unknown. We herein report a case with infracardiac type total anomalous pulmonary venous connection with remarkable oesophageal varices. In the present case, of note, the oesophageal varices were completely regressed after total anomalous pulmonary venous connection repair without any intervention. This case might help a surgical team reduce the hesitation to repair the total anomalous pulmonary venous connection regardless of oesophageal varices, a potentially fatal condition.

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.

Histopathology of anastomotic stenosis after total anomalous pulmonary vein connection.

We report a case of a 10-month-old girl who was diagnosed with pulmonary vein stenosis after total anomalous pulmonary vein connection repair and underwent release of an anastomotic stenosis. Histopathological examinations of the resected anastomotic tissue revealed intimal hyperplasia at the anastomotic site. Predominant lesion cells were identified as myofibroblasts and had the characteristics of fibroblasts and synthetic smooth muscle cells. These cells could be a useful target for preventing anastomotic stenosis after total anomalous pulmonary vein connection repair.

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.

Rare Case of Neonatal Cardiac Ischemia Due to Intermittent Aortic Insufficiency.

We report a case of a 23-day-old girl who presented with repeated life-threatening myocardial ischemia from intermittent aortic insufficiency because of fixation of the left coronary cusp against the aortic wall. The patient underwent aortic valve repair with partial commissuroplasty of the left-right and left-noncommissures. After the surgical intervention the patient has been well, with no ischemic event for over 1 year. This diagnosis should be considered as a differential diagnosis in pediatric cases with myocardial ischemia.

Progression of vascular remodeling in pulmonary vein obstruction.

OBJECTIVES: Pulmonary vein obstruction (PVO) frequently occurs after repair of total anomalous pulmonary vein connection with progression of intimal hyperplasia from the anastomotic site toward upstream pulmonary veins (PVs). However, the understanding of mechanism in PVO progression is constrained by lack of data derived from a physiological model of the disease, and no prophylaxis has been established. We developed a new PVO animal model, investigated the mechanisms of PVO progression, and examined a new prophylactic strategy. METHODS: We developed a chronic PVO model using infant domestic pigs by cutting and resuturing the left lower PV followed by weekly hemodynamic parameter measurement and angiographic assessment of the anastomosed PV. Subsequently, we tested a novel therapeutic strategy with external application of rapamycin-eluting film to the anastomotic site. RESULTS: We found the pig PVO model mimicked human PVO hemodynamically and histopathologically. This model exhibited increased expression levels of Ki-67 and phospho-mammalian target of rapamycin in smooth muscle-like cells at the anastomotic neointima. In addition, contractile to synthetic phenotypic transition; that is, dedifferentiation of smooth muscle cells and mammalian target of rapamycin pathway activation in the neointima of upstream PVs were observed. Rapamycin-eluting films externally applied around the anastomotic site inhibited the activation of mammalian target of rapamycin in the smooth muscle-like cells of neointima, and delayed PV anastomotic stenosis. CONCLUSIONS: We demonstrate the evidence on dedifferentiation of smooth muscle-like cells and mammalian target of rapamycin pathway activation in the pathogenesis of PVO progression. Delivery of rapamycin to the anastomotic site from the external side delayed PV anastomotic stenosis, implicating a new therapeutic strategy to prevent PVO progression.

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.

A novel diagnostic system for Doppler ultrasound of the peripheral arteries to detect the presence of thoracic aortic aneurysm.

OBJECTIVE: We aimed to develop a novel ultrasound system and examine its feasibility for noninvasively detecting thoracic aortic aneurysm (TAA) in clinical settings. METHODS: We developed a novel ultrasound system consisting of a modified console and data analysis algorithm. The exploratory study included 100 patients hospitalized for elective cardiovascular surgery. After admission, the arterial pulse waveform at the left carotid artery was acquired using the novel system. Based on these data, we inferred the presence of TAA based on arterial viscoelasticity and instability, which are reflected into the time-averaged trajectory of deformation of the blood vessel wall caused by disturbance of blood flow. Meanwhile, all patients underwent computed tomography as preoperative screening to confirm the presence of TAA. The sensitivity and specificity of TAA detection using the novel ultrasound system were calculated. RESULTS: The datasets from 37 patients were not suitable for analysis and were thus discarded. Based on computed tomography findings, 40 patients were categorized into the aneurysm group while 23 were judged not to have and aortic aneurysm. On the other hand, 44 patients were diagnosed as having TAA based on ultrasound findings obtained using the novel system. The overall sensitivity and specificity of the ultrasound system were 0.83 and 0.52, respectively. CONCLUSION: We successfully developed a novel system for noninvasive, ultrasound-based evaluation of the left carotid artery to detect TAA. Although improvements to the probe and diagnostic algorithm are warranted, this device has potential utility for mass screening to detect asymptomatic TAA as part of community-level healthcare programs.

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.

Suppressive effect of pitavastatin on aortic arch dilatation in acute stanford type B aortic dissection: analysis of STANP trial.

OBJECTIVES: Medical therapy for patients with uncomplicated acute type B aortic dissection (ABAD) is essentially accepted for its excellent early outcome; however, long-term outcomes have not been satisfactory due to aorta-related complications. This trial was performed to investigate the efficacy of a statin as an additive that may enhance the effectiveness of conventional medical treatment in patients with ABAD. METHODS: This was a multi-center, prospective, and randomized comparative investigation of patients with uncomplicated ABAD. Fifty patients with ABAD compatible with inclusion criteria were randomly assigned to two groups and then received administration of pitavastatin (group P) or not (group C). We followed up the patients for 1 year from study onset. RESULTS: Two patients demised during the follow-up period (both were in group C). In addition, aorta-related interventions were performed in two patients (entry closure for aortic dissection by endovascular repair in one patient in each group). Aortic arch diameters at 1 year in group P tended to be smaller than in group C (P = 0.17), and the rate of change of the aortic arch diameters from onset to 1 year was significantly lower in group P (P = 0.046). Multivariate analysis identified patency of the false lumen was detected as a risk factor for aortic arch dilatation (P = 0.02), and pitavastatin intake was a negative risk factor (P = 0.03). CONCLUSIONS: Pitavastatin treatment, in addition to the standard antihypertensive therapy, may have a suppressive effect on aortic arch dilatation in patients with ABAD.

Predictors of Heparin Resistance Before Cardiovascular Operations in Adults.

BACKGROUND: Heparin resistance (HR) is often encountered during cardiovascular operations that require cardiopulmonary bypass. Clinical risk factors and the mechanism underlying heparin resistance are yet to be determined. The aim of this study was to elucidate the clinically valid preoperative predictors related to HR. METHODS: The study evaluated 489 patients undergoing cardiovascular operations. Of these, 25 patients presented with HR and received antithrombin III for the initiation of cardiopulmonary bypass with an effective activated coagulation time. The remaining 464 patients, who did not receive antithrombin III, served as controls (NHR). Preoperative patient demographic and laboratory data were analyzed to identify risk factors for HR. RESULTS: The preoperative laboratory data showed platelet count, fibrinogen, D-dimer, creatinine, and C-reactive protein were significantly higher in the HR group than in the NHR group. As expected, the antithrombin III level was significantly lower overall in the HR group (86.0% vs 95.5%, p = 0.009); however, 80% of the patients in the HR group showed normal antithrombin III levels preoperatively. Multivariable logistic regression analysis identified chronic aortic dissection, chronic obstructive pulmonary disease, smoking, and elevated fibrinogen levels as independent predictors for HR. CONCLUSIONS: HR was shown to be associated with preoperative high fibrinogen levels, a smoking habit, and a preoperative diagnosis of chronic, but not acute, aortic dissection, with chronic obstructive pulmonary disease as comorbidity. Administration of antithrombin III resolved HR in all of the affected patients, even when their preoperative antithrombin III level was within the normal limit.

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.

Aortic Valve Replacement for Patients with Functioning Internal Mammalian Artery Grafts.

Aortic valve replacement (AVR) for patients with functioning internal mammalian artery (ITA) grafts is technically challenging, and the optimal treatment strategy for these situations remains controversial. Here, we report five cases of AVR with ITA graft using continuous retrograde cardioplegia in addition to moderate hypothermia without the clamping of ITA and discuss the management of these cases.

Oesophageal mucosal blood flow changes after thoracic endovascular stent graft implantation using a novel sensor probe.

OBJECTIVES: Secondary aorto-oesophageal fistula is a rare, lethal complication occurring after thoracic endovascular aneurysmal repair. The cause of secondary aorto-oesophageal fistula is unknown, but a reduction in local oesophageal mucosal blood flow (OMBF) may be a basis for such a devastating sequela. Our study aims to develop a novel blood flow sensor probe to detect changes in OMBF after thoracic stent graft implantation in an experimental swine model. METHODS: A novel laser Doppler flowmetry sensor probe incorporating an optical fibre sensor within a nasogastric tube was developed using microelectromechanical system technology. OMBF was measured at various levels using this sensor probe, to test its feasibility before and after thoracic endovascular stent graft implantation covering Th4-Th8 vertebral levels in 6 swine. RESULTS: In the middle oesophagus (Th5-Th7), where the aorta was covered with a stent graft, the measured OMBFs were significantly decreased after thoracic endovascular stent graft implantation than those of baseline (8.6 ± 2.7 vs 18.4 ± 7.9 ml/min/100 g, P < 0.0001), followed by a plateau period for at least 2 h after stent grafting (8.7 ± 3.3 ml/min/100 g, P < 0.0001 vs baseline). OMBFs in the upper (Th1-Th3) and lower (Th9-Th11) oesophagus, where the aorta was not covered with a stent graft, were unaffected by thoracic endovascular stent grafting. CONCLUSIONS: The novel laser Doppler flowmetry sensor probe was useful to monitor precise changes of OMBF in a swine model, demonstrating a significant reduction in OMBF after thoracic endovascular stent graft implantation.