[Selective Sinus Replacement for Aortic Root Disease].

BACKGROUND: Selective sinus replacement seems a reasonable option in cases requiring replacement of one or two sinuses of Valsalva, especially with acute aortic dissection and high bleeding risk. METHODS: Six patients (average age 58±17 years;five males) underwent selective replacement of the right sinus of Valsalva with right coronary artery bypass grafting (n=5) in 2015-2023. Five patients developed acute aortic dissection and one developed aneurysm of the right sinus of Valsalva. RESULTS: All patients survived the operation, and there were no cases requiring re-exploration for bleeding. Intraoperative transesophageal echocardiography showed trivial or less aortic regurgitation (AR) in all patients. Cardiopulmonary bypass time, aortic cross-clamping time, and lower body circulatory arrest time were 214±28 min, 159±22 min, and 31±6 min (n=5), respectively. During follow-up of 55±44 (4-104) months, all patients were asymptomatic. AR was mild or less in four patients, mild-moderate in one patient, and severe in one patient. All patients had normal cardiac function without left ventricular enlargement, and so no reoperation was required. CONCLUSIONS: Although this method appears to be relatively safe and effective, some patients developed late AR. Long-term follow-up of larger numbers of patients will be necessary to confirm its effectiveness.

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.

A Nationwide Survey of Surgical Treatment for Severe Ischemic Mitral Regurgitation.

OBJECTIVE: Mitral subvalvular procedures in addition to restrictive annuloplasty are promising for ischemic mitral regurgitation (IMR). However, the prevalence and efficacy of specific subvalvular repair in severe IMR have not been elucidated. This is the first nationwide survey regarding surgeons' attitudes toward IMR in Japan. METHODS: A questionnaire was sent to 543 institutions. From 2015 to 2019, numbers of elective first-time mitral valve replacement (MVR) with/without complete chordal preservation (CCP)/papillary muscle approximation (PMA) and mitral valvuloplasty (MVP) with/without papillary muscle relocation (PMR)/PMA in patients with severe IMR were collected. Concomitant procedures for coronary artery, tricuspid valve, and arrhythmia could be included but left ventricular reconstruction was excluded. RESULTS: Completed questionnaires were received from 286 institutions (52.7%). The majority (90%) had less than 20 cases within 5 years. The number of MVP (1413, 61.5%) surpassed MVR (886, 38.5%). CCP was performed in half of MVR (50.0%), while PMA was included in only 1.9% of MVR. PMA and PMR were also performed infrequently, in only 7.7% and 10.9% of MVP, respectively. CONCLUSION: Japanese surgeons aggressively perform MVP for severe IMR. Subvalvular repair was also aggressively performed in addition to MVR, but not to MVP. A multicenter registry study is in progress.

Aortic root destruction after aortic valvuloplasty for bicuspid aortic valve.

Ultrasound cardiography showed severe aortic regurgitation (AR) due to bicuspid aortic valve with dilatation of the aortic annulus and sinotubular junction in a 27-year-old man hospitalized with loss of consciousness. He underwent aortic valvuloplasty combined with external suture annuloplasty using an expanded polytetrafluoroethylene (ePTFE) suture. Intraoperative findings revealed thickening and adhesion of the aortic root despite the first surgery. He developed recurrent AR 7 months later and underwent redo surgery. An ePTFE suture was found inside the aorta. Aortic root replacement with a mechanical composite graft was performed, as reconstruction appeared difficult because the aortic annulus was damaged and there were multiple holes on all cusps. Here, we report a rare case of aortic root destruction after external suture annuloplasty.

Effect of cone reconstruction on right ventricular function in patients with Ebstein's anomaly: a meta-analysis.

OBJECTIVES: Cone reconstruction (CR) is a novel technique for surgically treating the tricuspid valve and right ventricle (RV) in patients with Ebstein's anomaly. However, precise changes in the RV function after CR remain unclear. This study aimed to evaluate the RV size and New York Heart Association (NYHA) functional class by conducting a meta-analysis of reported data. METHODS: The MEDLINE, EMBASE, Cochrane Library and China National Knowledge Infrastructure databases were searched for relevant studies. The variables were RV measurements, NYHA functional classes and tricuspid valve regurgitation grades. A fixed/random effects model was used to summarize the estimates of mean difference with standard error. Sensitivity analysis was conducted to ascertain the primary origin of the heterogeneity. RESULTS: Nine studies that involved 210 patients were included. The results demonstrated that after CR, the functional RV volume significantly decreased, NYHA functional class improved and tricuspid valve regurgitation grade decreased. CONCLUSIONS: Thus, CR appeared to be a positive approach for Ebstein's anomaly, with good results being obtained for the RV size and NYHA functional class. Because some limitations could not be overcome, studies with more data on RV and longer follow-ups are required to confirm our study results.

Tacrolimus-Induced Reversible Cerebral Vasoconstriction Syndrome with Delayed Multi-Segmental Vasoconstriction.

Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular syndrome characterized by multi-segmental constrictions of the cerebral arteries that resolves spontaneously within 3 months. Although RCVS is considered to be due to transient dysregulation of vascular tone, the exact pathomechanism remains unclear. We describe the case of a 15-year-old girl with RCVS induced by tacrolimus, who developed generalized seizure during the postoperative course of orthotropic heart transplantation. Magnetic resonance imaging at symptom onset showed a few vasoconstrictions accompanying brain edema and convexity subarachnoid hemorrhage. Although her neurological conditions rapidly improved after discontinuing tacrolimus, a repeat magnetic resonance angiogram demonstrated delayed progression of the multi-segmental vasoconstrictions followed by subsequent resolution. Our case demonstrates that cautious observation of the cerebral arteries using magnetic resonance angiography and careful management of vasoconstrictions with vasodilators are necessary for delayed vasoconstrictions even when the clinical symptoms improve.

Experiences With Aggressive Cardiac Rehabilitation in Pediatric Patients Receiving Mechanical Circulatory Supports.

Although some patients with fulminant myocarditis can be rescued owing to the improvements in mechanical circulatory support therapy, there are few reports providing evidence of cardiac rehabilitation during mechanical circulatory supports, particularly among pediatric patients. We treated two pediatric patients who underwent aggressive cardiac rehabilitation during mechanical support. Five days after the initiation of extracorporeal membrane oxygenation therapy aggressive cardiac rehabilitation was started in a 10-year-old girl with fulminant myocarditis. After explantation of the device, she was discharged on postoperative day 23. A 6-year-old girl with fulminant myocarditis started receiving cardiac rehabilitation two days after the initiation of an extracorporeal left ventricular assist device, despite having hemiplegia due to a recent broad stroke. She achieved an exercise capacity of supported walking for 280 meters after 127 days of cardiac rehabilitation and then went abroad to undergo heart transplantation when she was in the best physical condition possible. Early initiation of cardiac rehabilitation may be safe and effective for successful pediatric mechanical circulatory support therapy; this acts as a bridge to explantation or heart transplantation.

Superparamagnetic iron oxide nanoparticles function as a long-term, multi-modal imaging label for non-invasive tracking of implanted progenitor cells.

The purpose of this study was to determine the ability of superparamagnetic iron oxide (SPIO) nanoparticles to function as a long-term tracking label for multi-modal imaging of implanted engineered tissues containing muscle-derived progenitor cells using magnetic resonance imaging (MRI) and X-ray micro-computed tomography (µCT). SPIO-labeled primary myoblasts were embedded in fibrin sealant and imaged to obtain intensity data by MRI or radio-opacity information by µCT. Each imaging modality displayed a detection gradient that matched increasing SPIO concentrations. Labeled cells were then incorporated in fibrin sealant, injected into the atrioventricular groove of rat hearts, and imaged in vivo and ex vivo for up to 1 year. Transplanted cells were identified in intact animals and isolated hearts using both imaging modalities. MRI was better able to detect minuscule amounts of SPIO nanoparticles, while µCT more precisely identified the location of heavily-labeled cells. Histological analyses confirmed that iron oxide particles were confined to viable, skeletal muscle-derived cells in the implant at the expected location based on MRI and µCT. These analyses showed no evidence of phagocytosis of labeled cells by macrophages or release of nanoparticles from transplanted cells. In conclusion, we established that SPIO nanoparticles function as a sensitive and specific long-term label for MRI and µCT, respectively. Our findings will enable investigators interested in regenerative therapies to non-invasively and serially acquire complementary, high-resolution images of transplanted cells for one year using a single label.

Preliminary biomarkers for identification of human ascending thoracic aortic aneurysm.

BACKGROUND: Human ascending thoracic aortic aneurysms (ATAAs) are life threatening and constitute a leading cause of mortality in the United States. Previously, we demonstrated that collagens α2(V) and α1(XI) mRNA and protein expression levels are significantly increased in ATAAs. METHODS AND RESULTS: In this report, the authors extended these preliminary studies using high-throughput proteomic analysis to identify additional biomarkers for use in whole blood real-time RT-PCR analysis to allow for the identification of ATAAs before dissection or rupture. Human ATAA samples were obtained from male and female patients aged 65 ± 14 years. Both bicuspid and tricuspid aortic valve patients were included and compared with nonaneurysmal aortas (mean diameter 2.3 cm). Five biomarkers were identified as being suitable for detection and identification of ATAAs using qRT-PCR analysis of whole blood. Analysis of 41 samples (19 small, 13 medium-sized, and 9 large ATAAs) demonstrated the overexpression of 3 of these transcript biomarkers correctly identified 79.4% of patients with ATAA of ≥4.0 cm (P<0.001, sensitivity 0.79, CI=0.62 to 0.91; specificity 1.00, 95% CI=0.42 to 1.00). CONCLUSION: A preliminary transcript biomarker panel for the identification of ATAAs using whole blood qRT-PCR analysis in men and women is presented.

Transplantation of autologously derived mitochondria protects the heart from ischemia-reperfusion injury.

Mitochondrial damage and dysfunction occur during ischemia and modulate cardiac function and cell survival significantly during reperfusion. We hypothesized that transplantation of autologously derived mitochondria immediately prior to reperfusion would ameliorate these effects. New Zealand White rabbits were used for regional ischemia (RI), which was achieved by temporarily snaring the left anterior descending artery for 30 min. Following 29 min of RI, autologously derived mitochondria (RI-mitochondria; 9.7 ± 1.7 × 10(6)/ml) or vehicle alone (RI-vehicle) were injected directly into the RI zone, and the hearts were allowed to recover for 4 wk. Mitochondrial transplantation decreased (P < 0.05) creatine kinase MB, cardiac troponin-I, and apoptosis significantly in the RI zone. Infarct size following 4 wk of recovery was decreased significantly in RI-mitochondria (7.9 ± 2.9%) compared with RI-vehicle (34.2 ± 3.3%, P < 0.05). Serial echocardiograms showed that RI-mitochondria hearts returned to normal contraction within 10 min after reperfusion was started; however, RI-vehicle hearts showed persistent hypokinesia in the RI zone at 4 wk of recovery. Electrocardiogram and optical mapping studies showed that no arrhythmia was associated with autologously derived mitochondrial transplantation. In vivo and in vitro studies show that the transplanted mitochondria are evident in the interstitial spaces and are internalized by cardiomyocytes 2-8 h after transplantation. The transplanted mitochondria enhanced oxygen consumption, high-energy phosphate synthesis, and the induction of cytokine mediators and proteomic pathways that are important in preserving myocardial energetics, cell viability, and enhanced post-infarct cardiac function. Transplantation of autologously derived mitochondria provides a novel technique to protect the heart from ischemia-reperfusion injury.

Homograft use in patient with tetralogy of Fallot and absent pulmonary valve.

A neonate with tetralogy of Fallot and absent pulmonary valve presented with respiratory distress due to airway obstruction after birth. He was placed on mechanical respiration. At 28-days old, he underwent pulmonary artery reduction and pulmonary homograft insertion. Ventricular septal defect patch closure was performed under cardiopulmonary bypass. His postoperative course was complicated by prolonged mechanical ventilatory support because of tracheomalacia. After 1 year of follow-up, he was in good condition without pulmonary regurgitation.

Neonatal repair of right interrupted aortic arch with cerebro-myocardial perfusion technique.

Right interrupted aortic arch and descending aorta is exceedingly rare and most likely cause respiratory presentation, since patent ductus arteriosus (PDA) courses over the right mainstem bronchus. We report a case of successful neonatal biventricular repair of a right interrupted aortic arch (type B), with an aberrant right subclavian artery ventricular septal defect (VSD) in a 2.7 kg term neonate with DiGeorge syndrome. Patient presented in severe respiratory distress and acidosis at one day old. Two-dimensional (2D) echocardiography revealed aortic arch interruption beyond the common carotid arteries with large perimembranous outlet VSD. Aortic annulus diameter was 4.8 mm and there was no left ventricle (LV) outflow tract obstruction. Three-dimensional (3D) CT-scan confirmed these findings and identified a right-sided ductal arch that continued over the right mainstem bronchus into a right-sided descending aorta and aberrant right subclavian artery. Brachiocephalic perfusion and ductal perfusion was employed for cooling during cardiopulmonary bypass. Under deep hypothermia (27 °C rectal temperature), selective cerebro-myocardial perfusion was used for successful aortic arch repair without sacrificing the aberrant right subclavian artery. A direct tension-free anastomosis was attained. Her postoperative course was uneventful and her respiratory symptoms disappeared postoperatively. Early surgical correction is mandatory for these patients with unique anatomy and presentation.

One-Stage Surgical Repair for Berry Syndrome With Preoperative Diagnosis by 3-Dimensional CT.

Berry syndrome is a rare congenital combination of an aortopulmonary window, an aortic origin of the right pulmonary artery, an interrupted aortic arch with a patent ductus arteriosus, and an intact ventricular septum. We report a successful one-stage surgical correction of Berry syndrome. Also, we demonstrate the importance of prompt clinical recognition with echocardiography and 3-dimensional reconstruction of computed tomography (3D-CT) and timely operation for the management of this rare cardiac anomaly.

Image-guided surgical repair of ventricular septal rupture using self-expanding device.

The purpose of this study was to determine the possibility of a new surgical technique for the treatment of ventricular septal rupture after acute myocardial infarction. The operations were conducted under the guidance of real-time three-dimensional echocardiography (RT3DE) (iE33, Philips Medical Systems, Andover, MA). Six pigs were anesthetized, and after median sternotomy, the echo probe was applied directly to the surface of the heart. A ventricular septal defect (VSD) was created in all the six porcine hearts. The VSDs were closed with an Amplatzer septal occluder (AGA Medical Corp, Golden Valley, MN) through the right ventricular free wall under RT3DE monitoring. The procedure was successful in all the six pigs. The VSDs were precisely closed with the septal occluder under RT3DE guidance. Both left ventriculography and color-Doppler echocardiography showed no residual shunt in any of the six pigs. The use of the Amplatzer septal occluder under the guidance of real-time three-dimensional echocardiography made it possible to close the ventricular septal defect safely and successfully without the need to resort to cardiopulmonary bypass. Our results strongly suggest that the application of this new technique is feasible for the treatment of ventricular septal rupture after acute myocardial infarction.

In early-stage diabetic retinopathy, risk of cardiac events after implantation of sirolimus-eluting stent is higher than after coronary artery bypass surgery.

BACKGROUND: Patients with diabetic retinopathy (DR) have an increased risk of death from coronary heart disease and myocardial infarction. The purpose of this study was to compare the outcomes of revascularization strategies (sirolimus-eluting stent [SES] and coronary artery bypass surgery [CABG]) in patients with DR according to the stage of retinopathy: non-proliferative retinopathy (NPDR) and proliferative retinopathy (PDR). METHODS: From April 2004 until February 2007, 627 patients including 51 NPDR and 62 PDR patients underwent SES implantation. For each retinopathy group, a historical comparison group at the same stages of retinopathy undergoing CABG was selected. Cardiac events were defined as a composite of cardiac death, myocardial infarction, and repeat revascularization. RESULTS: The average follow-up from the time of the initial revascularization was 27.7 ± 8.5 months for NPDR-SES patients, 69.6 ± 36.6 months for NPDR-CABG patients, 26.4 ± 9.7 months for PDR-SES patients, and 68.3 ± 44.2 months for PDR-CABG patients; and Kaplan-Meier estimates of the percentages of events at 24 months were 47.0%, 22.8%, 28.5%, and 26.0%. Kaplan-Meier curves for cardiac events differed significantly between the SES group and the CABG group in NPDR patients (p = 0.04), whereas the curves did not differ significantly between the two groups of PDR patients. The adjusted hazard ratio of SES implantation for cardiac events in the entire group of DR patients was 1.75 (95% confidence interval [CI] 1.02-3.00, p = 0.04). CONCLUSIONS: SES implantation is not a suitable method of revascularization in DR patients, especially in NPDR patients. CABG may become the first-choice revascularization technique for these patients.

Bronchial migration of a systemic-pulmonary shunt conduit.

Using an expanded polytetrafluoroethylene conduit, a subclavian artery-to-pulmonary artery shunt was created in an infant with tetralogy of Fallot. The postoperative course was complicated by sepsis, shunt occlusion, and pneumonia. Four years later, an obstructive mass was found in the right main bronchus on fluoroscopy and was retrieved on rigid endoscopy, which turned out to be the migrated conduit. This case implies that a vascular conduit anastomosed to a systemic artery can migrate into the airway without bleeding, pseudoaneurysm formation, or host artery occlusion.