Novel Technique for Median Cleft Lip Comprising the Simultaneous Formation of the Columella, Philtrum, and Cupid's Bow.

Numerous surgical techniques for median cleft lip repair have been described; however, most cause excessively sharp peaks or the collapse of Cupid's bow. We report a technique for median cleft lip repair using a mucosal skin flap and full-thickness skin graft and 15 years of follow-up. Our technique provides acceptable formation of the columella, philtrum, and the two peaks of Cupid's bow. In this paper, we cite our previously reported techniques and add new findings and discussion based on the long-term postoperative outcomes of this procedure. Advantages and disadvantages of this technique are discussed, and a possible solution to achieve a more satisfactory result is suggested. Advantages and disadvantages of this new technique are discussed, and a possible solution to achieve a more satisfactory result is suggested.

Re-expansion pulmonary edema after minimally invasive cardiac surgery in children.

Re-expansion pulmonary edema is a serious complication that can occur after minimally invasive cardiac surgery through a right mini-thoracotomy. Herein, we describe two paediatric cases where re-expansion pulmonary edema was observed after simple atrial septal defect closure through a right mini-thoracotomy. This is the first case report of re-expansion pulmonary edema after a paediatric cardiac surgery.

Risk factors of local recurrence following implant-based breast reconstruction in breast cancer patients.

BACKGROUND: The number of patients desiring implant-based breast reconstruction has been increasing. While local recurrence is observed in patients with breast reconstruction, only a few reports have focused on the risk factors for local recurrence and the prognosis after developing local recurrence. METHODS: We analyzed 387 patients who underwent implant-based breast reconstruction during the period from 2004 to 2017 in Hiroshima City Hospital. We retrospectively examined the risk factors for local recurrence and the outcomes of patients developing such recurrence after implant-based breast reconstruction. RESULTS: The median follow-up time was 59 months. The local recurrence rate was 3.1% (n = 12). The most common reason for detecting local recurrence was a palpable mass. Four patients with local recurrence had recurrence involving the skin just above the primary lesion and needle biopsy tract. All patients with local recurrence received surgery and systemic therapy and most patients received radiation therapy, all have remained free of new recurrence to date. Multivariate analysis showed lymphatic vessel invasion (HR, 6.63; 95% CI, 1.40-31.36; p = 0.017) and positive or < 2 mm vertical margin (HR, 9.72; 95%CI, 1.23-77.13; p = 0.047) to be associated with significantly increased risk of local recurrence. CONCLUSIONS: The risk factors for local recurrence following implant-based breast reconstruction were lymphatic vessel invasion and positive or < 2 mm vertical margin. Removal of the skin just above the primary lesion and needle biopsy tract and adjuvant radiation therapy might improve local outcomes. Patients with local recurrence following implant-based breast reconstruction appear to have good outcomes with appropriate treatment.

Dysphagia Aortica Caused by Congenitally Angulated Descending Aorta.

Dysphagia aortica is a rare pathology primarily caused by an aortic aneurysm or Kommerrell's diverticulum. Herein, we describe an extremely rare case of dysphagia aortica due to a congenitally angulated descending aorta in a 50-year-old woman successfully treated by open surgery. The woman underwent David procedure for aortic regurgitation 5 years previously, with the same anatomy of an angulated descending aorta without symptoms. She has difficulty in swallowing solid food from 1 month. Total aortic arch replacement using the elephant trunk technique and secondary descending aortic replacement released the esophageal compression by the abnormal aorta and completely improved her symptom.

Outcomes of pulmonary valve replacement using a bioprosthetic valve and polytetrafluoroethylene bicuspid valve for pulmonary valve regurgitation after intracardiac repair of tetralogy of Fallot.

BACKGROUND: Excellent results have been reported regarding pulmonary valve replacement (PVR) for pulmonary valve regurgitation (PR) after intracardiac repair (ICR) in patients with tetralogy of Fallot (TOF). However, there are apparently no reports comparing the following procedures: PVR using a bioprosthetic valve and PVR using a polytetrafluoroethylene (PTFE) bicuspid valve. Herein, we retrospectively analyzed the outcomes of PVR for PR after ICR of TOF in our institution and assessed which of the two PVR procedures was better. METHODS: From June 2008 to December 2017, we performed PVR for PR after ICR of TOF in 34 patients. Patients with the right ventricle to the pulmonary artery conduits were excluded. Preoperative and postoperative cardiac magnetic resonance imagings (cMRIs) were performed in all patients. The patients were divided into the bioprosthetic valve group (BV group, n = 17) and the PTFE bicuspid valve group (PTFE group, n = 17). RESULTS: There were no significant differences in the preoperative cMRI data and perioperative factors between the two groups. There were no deaths in either group. Postoperative cMRI showed that the PR fraction and the right ventricular end-diastolic volume index (RVEDVI)/left ventricular end-diastolic volume index ratio were significantly improved in both groups. However, RVEDVI was significantly improved only in the BV group. Re-PVR was required in four patients in the PTFE group. CONCLUSION: PVR using a bioprosthetic valve was more effective for PR treatment after ICR of TOF than PVR using a PTFE bicuspid valve.

Pulmonary valve replacement after tetralogy of Fallot repair in a patient with immune thrombocytopenia.

Marked thrombocytopenia causes significant bleeding in cardiovascular surgery. Herein, we describe the case of a 47-year-old woman with immune thrombocytopenia who underwent successful pulmonary valve replacement for pulmonary valve regurgitation and stenosis after complete repair of tetralogy of Fallot. Her platelet count decreased significantly to less than 5 × 109 /L on postoperative day 3, thus multiple platelet transfusions were given. Pulse steroid therapy with dexamethasone was subsequently administered systemically for 4 days. After the treatment, her platelet count started to recover. There were no significant postoperative bleeding events, and red blood cell transfusion was not required. Other than the platelet event, the postoperative course was uneventful and the patient was discharged on postoperative day 15.

Superior trans-septal approach for minimally invasive mitral valve surgery via right small thoracotomy.

Minimally invasive mitral valve surgery (MIMVS), despite its challenges, is not a rare procedure. However, MIMVS via a right small thoracotomy must be performed using long-shafted surgical instruments and thoracotomy instruments specialized for minimally invasive cardiac surgeries. We have performed 12 cases of MIMVS via right small thoracotomy using the superior trans-septal approach and secured a surgical visual field that easily allows a finger to reach the mitral valve annulus without using special instruments for minimally invasive cardiac surgery. We named this technique the "drawer-case technique." In conclusion, MIMVS via right thoracotomy using the superior trans-septal approach can be performed easily and safely, similar to mitral valve surgery performed via median sternotomy.

Computed Fluid Dynamics Analysis for SVC-to-RPA Anastomosis With Antegrade Pulmonary Flow.

We report an infant case after superior vena cava -to-right pulmonary artery anastomosis with antegrade pulmonary flow in which computational fluid dynamics analysis showed that restriction of antegrade blood flow by the remaining right pulmonary stenosis resulted in reduced shear stress and energy loss in the superior vena cava.

Chondromodulin-I maintains cardiac valvular function by preventing angiogenesis.

The avascularity of cardiac valves is abrogated in several valvular heart diseases (VHDs). This study investigated the molecular mechanisms underlying valvular avascularity and its correlation with VHD. Chondromodulin-I, an antiangiogenic factor isolated from cartilage, is abundantly expressed in cardiac valves. Gene targeting of chondromodulin-I resulted in enhanced Vegf-A expression, angiogenesis, lipid deposition and calcification in the cardiac valves of aged mice. Echocardiography showed aortic valve thickening, calcification and turbulent flow, indicative of early changes in aortic stenosis. Conditioned medium obtained from cultured valvular interstitial cells strongly inhibited tube formation and mobilization of endothelial cells and induced their apoptosis; these effects were partially inhibited by chondromodulin-I small interfering RNA. In human VHD, including cases associated with infective endocarditis, rheumatic heart disease and atherosclerosis, VEGF-A expression, neovascularization and calcification were observed in areas of chondromodulin-I downregulation. These findings provide evidence that chondromodulin-I has a pivotal role in maintaining valvular normal function by preventing angiogenesis that may lead to VHD.

Molecular mechanisms underlying the onset of degenerative aortic valve disease.

Morbidity from degenerative aortic valve disease is increasing worldwide, concomitant with the ageing of the general population and the habitual consumption of diets high in calories and cholesterol. Immunohistologic studies have suggested that the molecular mechanism occurring in the degenerate aortic valve resembles that of atherosclerosis, prompting the testing of HMG CoA reductase inhibitors (statins) for the prevention of progression of native and bioprosthetic aortic valve degeneration. However, the effects of these therapies remain controversial. Although the molecular mechanisms underlying the onset of aortic valve degeneration are largely unknown, research in this area is advancing rapidly. The signaling components involved in embryonic valvulogenesis, such as Wnt, TGF-beta(1), BMP, and Notch, are also involved in the onset of aortic valve degeneration. Furthermore, investigations into extracellular matrix remodeling, angiogenesis, and osteogenesis in the aortic valve have been reported. Having noted avascularity of normal cardiac valves, we recently identified chondromodulin-I (chm-I) as a crucial anti-angiogenic factor. The expression of chm-I is restricted to cardiac valves from late embryogenesis to adulthood in the mouse, rat, and human. In human degenerate atherosclerotic valves, the expression of vascular endothelial growth factor (VEGF) and matrix metalloproteinases and angiogenesis is observed in the area of chm-I downregulation. Gene targeting of chm-I resulted in VEGF expression, angiogenesis, and calcification in the aortic valves of aged mice, and aortic stenosis is detected by echocardiography, indicating that chm-I is a crucial factor for maintaining normal cardiac valvular function by preventing angiogenesis. The present review focuses on the animal models of aortic valve degeneration and recent studies on the molecular mechanisms underlying the onset of degenerative aortic valve disease.

No-touch saphenous vein graft harvesting technique for coronary artery bypass grafting.

OBJECTIVES: Although the patency of internal artery grafts in coronary artery bypass grafting (CABG) is superior to that of saphenous vein grafts (SVGs), good long-term patency rates have been reported with SVGs harvested using the no-touch technique. Here, we report the early results of CABG with no-touch SVGs (NT-SVGs) along with the precautions required while handling these grafts. METHODS: This retrospective study included 41 patients who underwent CABG with NT-SVGs between May 2016 and March 2018. NT-SVGs were harvested with minimal electric scalpel use, and post-harvesting vascular overdilation with a syringe was not performed. Blood with heparinized saline was used to prevent vascular endothelial damage. A drain was inserted into the SVG harvest site, which was closed with a single interrupted suture, and elasticated bandage and compression stockings were used to prevent fluid retention and avoid delayed wound healing. RESULTS: There was no case of postoperative thoracotomy due to bleeding or myocardial infarction, and postoperative graft assessment confirmed graft patency in all patients, with a little mismatch between the diameters of SVG and the anastomotic vessel. Delayed wound healing at the graft harvest site seemed to improve after introducing the above procedures. CONCLUSIONS: Early results of CABG with NT-SVGs were good. Some precautions must be taken during harvesting and anastomosis. Taking precautions described in this study may help reduce the risk of perioperative complications and make the no-touch technique the standard procedure for harvesting SVGs for CABG.

Local tenomodulin absence, angiogenesis, and matrix metalloproteinase activation are associated with the rupture of the chordae tendineae cordis.

BACKGROUND: Rupture of the chordae tendineae cordis (CTC) is a well-known cause of mitral regurgitation. Despite its importance, the mechanisms by which the CTC is protected and the cause of its rupture remain unknown. CTC is an avascular tissue. We investigated the molecular mechanisms underlying the avascularity of CTC and the correlation between avascularity and CTC rupture. METHODS AND RESULTS: We found that tenomodulin, which is a recently isolated antiangiogenic factor, was expressed abundantly in the elastin-rich subendothelial outer layer of normal rodent, porcine, canine, and human CTC. Conditioned medium from cultured CTC interstitial cells strongly inhibited tube formation and mobilization of endothelial cells; these effects were partially inhibited by small-interfering RNA against tenomodulin. The immunohistochemical analysis was performed on 12 normal and 16 ruptured CTC obtained from the autopsy or surgical specimen. Interestingly, tenomodulin was locally absent in the ruptured areas of CTC, where abnormal vessel formation, strong expression of vascular endothelial growth factor-A and matrix metalloproteinases, and infiltration of inflammatory cells were observed, but not in the normal or nonruptured area. In anesthetized open-chest dogs, the tenomodulin layer of tricuspid CTC was surgically filed, and immunohistological analysis was performed after several months. This intervention gradually caused angiogenesis and expression of vascular endothelial growth factor-A and matrix metalloproteinases in the core collagen layer in a time-dependent manner. CONCLUSIONS: These findings provide evidence that tenomodulin is expressed universally in normal CTC in a concentric pattern and that local absence of tenomodulin, angiogenesis, and matrix metalloproteinase activation are associated with CTC rupture.

Regenerative Therapy for Patients with Congenital Heart Disease.

Congenital heart disease (CHD) is the most common birth defect, affecting 1 in 100 babies. Among CHDs, single ventricle (SV) physiologies, such as hypoplastic left heart syndrome and tricuspid atresia, are particularly severe conditions that require multiple palliative surgeries, including the Fontan procedure. Although the management strategies for SV patients have markedly improved, the prevalence of ventricular dysfunction continues to increase over time, especially after the Fontan procedure. At present, the final treatment for SV patients who develop heart failure is heart transplantation; however, transplantation is difficult to achieve because of severe donor shortages. Recently, various regenerative therapies for heart failure have been developed that increase cardiomyocytes and restore cardiac function, with promising results in adults. The clinical application of various forms of regenerative medicine for CHD patients with heart failure is highly anticipated, and the latest research in this field is reviewed here. In addition, regenerative therapy is important for children with CHD because of their natural growth. The ideal pediatric cardiovascular device would have the potential to adapt to a child's growth. Therefore, if a device that increases in size in accordance with the patient's growth could be developed using regenerative medicine, it would be highly beneficial. This review provides an overview of the available regenerative technologies for CHD patients.

In Vivo Remodeling of an Extracellular Matrix Cardiac Patch in an Ovine Model.

Lack of an ideal patch material for cardiac repairs continues to challenge congenital heart surgeons. The current materials are unable to grow and result in scarring, contraction, and arrhythmias. An acellular extracellular matrix (ECM) patch derived from porcine small intestinal submucosa has demonstrated remodeling potential when used to repair various tissues. This study investigated the in vivo electrophysiologic, mechanical, and histological properties of an ECM patch used to repair a right-ventricular (RV) wall defect in a growing ovine model. A full-thickness, 2 × 2 cm RV defect was created in 11 juvenile sheep and repaired with an ECM patch. Longitudinal RV three-dimensional-electrical mapping, magnetic resonance imaging (MRI), and histological analysis were performed at 3, 6, 9, and 12 months. Three-dimensional mapping demonstrated consistent conduction across the patch with little to no difference in voltage, but conduction velocity was consistently less than native myocardium. Magnetic resonance imaging revealed changing strain properties of the patch which by 9-12 months resembled native tissue. Histologic analysis at 3 months demonstrates cardiomyocyte degeneration and partial replacement via proliferation of connective tissue cells that were predominately fibroblasts and smooth muscle cells. There was marked neovascularization and an absence of calcification at 12 months. Over time, the ECM patch remained viable with stable muscle at the edges. In growing sheep, an ECM patch becomes a viable tissue and remains so up to at least a year. Although ECM demonstrates some functional aspects of remodeling to native myocardium, histologically it remained immature.

Diagnostic Performance of a Temporal Artery Biopsy for the Diagnosis of Giant Cell Arteritis in Japan-A Single-center Retrospective Cohort Study.

Objectives To investigate the sensitivity and specificity of a temporal artery biopsy (TAB) in the diagnosis of giant cell arteritis (GCA) in a single-center retrospective cohort in Japan. Methods A retrospective chart review was performed on consecutive patients who visited our hospital between April 2009 and October 2018 and underwent a TAB. The sensitivity and specificity were calculated for the three pathological standards for a TAB, predetermined according to the pathological criterion of the 1990 American College of Rheumatology (ACR) criteria: A) vasculitis characterized by predominant mononuclear cell infiltration; B) vasculitis with granulomatous inflammation; and C) vasculitis with multinucleated giant cells. We also analyzed the clinical parameters predicting the diagnosis of GCA and the impact of a diagnostic delay of ≥3 months on cardiovascular complications of GCA. Results Our study population was 16 cases in the GCA group and 13 in the non-GCA group. The sensitivity and specificity for Standard A of a TAB were 81% and 85%, respectively, while those for stricter Standards B or C were identical, at 75% and 100%, respectively. These pathological standards, but not any other parameters, significantly predicted the diagnosis. A diagnostic delay tended to cause cardiovascular complications (p=0.057). Conclusion The sensitivity and specificity of the pathological standards of a TAB were favorable in our cohort and were the only predictors for the diagnosis of GCA. Considering the possible impact of a diagnostic delay on cardiovascular complications, the early recognition and prompt initiation of glucocorticoid therapy is needed, even in Japan, where GCA is uncommon.

Thoraco-abdominal aortic aneurysm rupture in a patient with Shprintzen-Goldberg syndrome.

Shprintzen-Goldberg syndrome is a rare systemic connective tissue disorder characterized by craniosynostosis, skeletal abnormalities, infantile hypotonia, mild-to-moderate intellectual disability and cardiovascular anomalies. To our knowledge, this is the first report of a Shprintzen-Goldberg syndrome patient who developed a thoraco-abdominal aortic aneurysm. The aneurysm grew rapidly necessitating emergent thoraco-abdominal aortic replacement. The postoperative course was uneventful, and a careful lifetime follow-up was planned.

Stonehenge technique is associated with faster aortic clamp time in group of minimally invasive aortic valve replacement via right infra-axillary thoracotomy.

BACKGROUND: Trans-right axillary aortic valve replacement (TAX-AVR) remains uncommon. We developed a special method to pull the heart closer to the right chest wall to make the surgery as easy and safe as aortic valve replacement via median sternotomy. Because the retraction sutures lifting the ascending aorta and aortic root are arranged circularly around the wound, we named this technique "Stonehenge technique". METHODS: We examined 47 patients who underwent aortic valve replacement through a small right infra-axillary thoracotomy as the initial surgical therapy. These patients were divided into two groups: the conventional TAX-AVR group that underwent AVR via the conventional small right axillary incision approach (n = 20) and the TAX-AVR with SH group that underwent AVR with the Stonehenge technique (n = 27). RESULTS: The aortic cross-clamp and the extracorporeal circulation time were significantly shorter in the TAX-AVR with SH group than in the conventional TAX-AVR group (conventional TAX-AVR group: 125.5 ± 47.9; TAX-AVR with SH group: 96.0 ± 14.0, p = 0.004, and conventional TAX-AVR group: 163.8 ± 55.9; TAX-AVR with SH group: 140.0 ± 16.8, p = 0.04). CONCLUSION: The outcomes of this technique depend on the site of the retraction sutures in the opened pericardium, direction of pull, amount of force applied, and precautions taken. If performed correctly, the ascending aorta and the root can be pulled from the wound to within the surgeon's fingers' reach, thereby reducing aortic cross-clamp and extracorporeal circulation times in group of minimally invasive aortic valve replacement via right infra-axillary thoracotomy.

Thoracic Endovascular Aortic Repair for Pseudoaneurysm after Interrupted Aortic Arch Repair.

Here we describe the case of a 33-year-old woman who was diagnosed with interrupted aortic arch (IAA) type A and who underwent radical surgery in her infancy. She developed a 42-mm anastomotic pseudoaneurysm in the distal aortic arch. We decided to perform thoracic endovascular aortic repair because of the patient's special request to avoid open surgery. We selected a reversed taper-type leg stent graft for the iliac artery and successfully implanted it without problems. However, the long-term outcomes of the stent graft in young people remain unclear, and careful regular follow-up for a long period is mandatory.

Left atrioventricular valve replacement in a patient with hereditary haemorrhagic telangiectasia.

Hereditary haemorrhagic telangiectasia, or Osler-Rendu-Weber disease, is characterized by multiple arteriovenous malformations and telangiectasia which develop in the brain, lungs or liver. We report the case of a patient who had severe left atrioventricular valve regurgitation with atrial fibrillation after atrioventricular septal defect repair in her childhood and was diagnosed as having hereditary haemorrhagic telangiectasia by chance. The patient underwent successful left atrioventricular valve replacement and maze operation. The postoperative course was uneventful without nose bleeding worsening.