[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.

Right-to-left shunt via iatrogenic atrial septal defect requiring emergency surgical repair following cryoballoon atrial fibrillation ablation in a patient with arrhythmogenic right ventricular cardiomyopathy.

An 81-year-old woman with arrhythmogenic right ventricular cardiomyopathy underwent catheter ablation for atrial fibrillation and atrial flutter. Hypoxemia refractory to the administration of oxygen was seen after transseptal puncture. Transthoracic echocardiography revealed right to left shunt via an iatrogenic atrial septal defect (IASD) that was increased by tricuspid regurgitation flow. Her hypoxemia improved after IASD occlusion with the inflation of a venogram balloon catheter. Emergent surgical IASD closure was successfully performed. IASD after transseptal puncture for atrial fibrillation ablation infrequently causes severe complications that require emergent repair. Learning objective: Some cases requiring iatrogenic atrial septal defect (IASD) closure after atrial fibrillation (AF) ablation have been reported. We describe the case of an arrhythmogenic right ventricular cardiomyopathy patient with right to left shunt via an IASD which required emergent surgical repair after AF ablation. Right to left shunt after trans-septal puncture is rare, however it can be an emergent life-threatening complication. IASD occlusion with venogram balloon catheter is helpful for the diagnosis and the short-term solution.

Optimal threshold score of aortic valve calcification for identification of significant aortic stenosis on non-electrocardiographic-gated computed tomography.

OBJECTIVES: This study evaluated the association between aortic valve calcification (AVC) and aortic stenosis (AS) by scoring the AVC to determine the threshold scores for significant AS on non-electrocardiographic (ECG)-gated computed tomography (CT). METHODS: We retrospectively analyzed the AVC scores of 5385 patients on non-contrast non-ECG-gated CT, who underwent transthoracic echocardiography (TTE) from March 1, 2013, to December 26, 2019, at our institution. Multivariable logistic regression models were used to identify potential risk factors for significant AS. The thresholds for significant AS were computed using receiver operator characteristic (ROC) curves, based on the AVC scores after propensity score matching. RESULTS: A significant association was found between AS and age (p < 0.001; odds ratio [OR], 1.04; 95% confidence interval [CI], 1.02-1.06), female sex (p < 0.001; OR, 4.5; 95% CI, 2.75-7.36), bicuspid aortic valve (p < 0.001; OR, 23.2; 95% CI, 7.35-72.9), and AVC score (AVC score/100) (p < 0.001; OR, 1.82; 95% CI, 1.71-1.95). All sex-specific AVC thresholds for significant AS (moderate and over AS severity, moderate and over AS severity without discordance, discordant severe AS, and concordant severe AS) showed high sensitivity and specificity (AUC, 0.939-0.968; sensitivity, 84.6-96%; specificity, 84.2-97.1%). CONCLUSIONS: We determined the optimal AVC threshold scores for significant AS, which may aid in diagnosing significant asymptomatic AS on incidental detection of AVC through non-ECG-gated CT for non-cardiac indications. KEY POINTS: • Increased frequency of non-electrocardiographic (ECG)-gated computed tomography (CT) for non-cardiac indications has led to the increased incidental identification of aortic valve calcification (AVC). • It is important to identify patients with significant aortic stenosis (AS) who require additional echocardiographic assessment on incidental detection of AVC via non-ECG-gated CT. • We determined the AVC thresholds with high sensitivity and specificity to identify significant AS on non-ECG-gated CT, which could lead to early diagnosis of asymptomatic significant AS and improved prognosis.

A case of cardiac strangulation following epicardial pacemaker implantation in an adult: a case report.

Background: Cardiac strangulation (CS) is a rare but potentially devastating complication caused by the leads of an epicardial pacemaker (EP). Most cases have been reported in paediatric patients, and there has been no report wherein the diagnosis was made in a living, adult patient, and treated successfully. Case summary: A 31-year-old woman with a history of atrial septal defect (ASD) patch closure and EP implantation for congenital atrial stand-still presented with dyspnoea on exertion. The blood investigation of the patient showed liver dysfunction, chest radiography showed pulmonary artery dilatation, and transthoracic echocardiography showed right chambers dysfunction. Right heart catheterization showed haemodynamics similar to those of constrictive pericarditis, eventually leading to the diagnosis of CS due to EP leads. The patient was successfully operated upon. Discussion: We reported the first case where CS was diagnosed in adulthood and successfully treated with surgical intervention. Cardiac strangulation is challenging to diagnose because of the small number of cases reported and the lack of definitive diagnostic algorithms or criteria. Surgical EP lead removal should be performed without hesitation in cases where CS is considered the primary aetiology of critical symptoms or complications because surgical removal is the only fundamental treatment for CS. In addition, paediatric patients undergoing EP implantation need for close follow-up.

Constrictive Pericarditis with Cardiac Ascites Caused Spontaneous Bacterial Peritonitis.

Patients with constrictive pericarditis (CP) typically present with symptoms related to right-sided heart failure, such as cardiac ascites. Spontaneous bacterial peritonitis (SBP) usually arises in association with ascites secondary to hepatic cirrhosis. We herein report a rare case of CP in which SBP developed due to cardiac ascites, even in the absence of cirrhosis. In this case, pericardiectomy improved both the hemodynamics and the ascites, while therapy with diuretics alone was insufficient. It is important to consider SBP in the differential diagnosis when any abdominal symptoms or an inflammatory response is found in patients with heart failure and cardiac ascites.

Successful Repair of a Forme Fruste Bicuspid Aortic Valve by the Raphe Suspension Technique.

A 52-year-old man underwent surgery due to shortness of breath caused by severe aortic regurgitation with right coronary cusp prolapse. Operative findings revealed 3 symmetric cusps with small raphe between the right and noncoronary cusps situated lower than the others, indicating a forme fruste bicuspid aortic valve (BAV). The BAV was successfully repaired by tricuspidization, including raphe suspension, right coronary cusp plication, and double annuloplasty. The postoperative course was uneventful, and echocardiography at 3 months showed mild aortic regurgitation with adequate left ventricular reverse remodeling. Here we present the technical details of the raphe suspension procedure for forme fruste BAV.

Pulmonary Artery Intimal Sarcoma Diagnosed Preoperatively by Endovascular Biopsy and Treated via Right Pneumonectomy and Pulmonary Arterioplasty.

Introduction. Intimal sarcoma is a very rare tumor arising within the intima of the pulmonary artery. Preoperative diagnosis of pulmonary artery sarcoma is difficult, and the tumor is sometimes misdiagnosed as pulmonary thromboembolism. We report a case of pulmonary artery intimal sarcoma successfully diagnosed by preoperative endovascular biopsy and treated via right pneumonectomy and pulmonary arterioplasty. Presentation of a Case. A 72-year-old woman was referred to our hospital with a low-attenuation defect in the lumen of the right main pulmonary artery by computed tomography. Pulmonary artery thromboembolism was suspected, and anticoagulation therapy was administered. However, the defect in the pulmonary artery did not improve. Endovascular catheter aspiration biopsy was performed. Histological examination revealed pulmonary artery sarcoma. The patient was treated with right pneumonectomy and arterioplasty with the use of cardiopulmonary bypass. Discussion. Preoperative biopsy by endovascular catheter is worth considering for a patient with a tumor in the pulmonary artery and can help in planning treatment strategies.

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.

Safe Use of Glutaraldehyde to Repair the Destroyed Valve in Active Infective Mitral Valve Endocarditis.

BACKGROUND: The aim of this study was to review our experience of mitral valve (MV) repair for acute and active infective endocarditis (AAIE) and to identify the feasibility of a new approach together with the mid-term results. Methods and Results: A retrospective analysis was performed on 35 consecutive AAIE patients surgically treated in the isolated mitral position. Mean follow-up after the surgery was 4.3±3.7 years. 30 of the 35 patients were successfully treated by MV plasty (MVP); however, MV replacement (MVR) was necessary in the remaining 5 patients. Our novel approach included resection of the infective lesion, approximation with direct suture and/or patch repair with bovine or autopericardium after 2-min treatment of it and the defective leaflet edge(s) with 0.625% glutaraldehyde solution, reconstruction with artificial chordae and ring annuloplasty. The success rate of MVP was 85.7%. The longest postoperative follow-up echocardiography showed no mitral regurgitation (MR) in 4, trivial MR in 4, mild MR in 16 and moderate MR in 5 patients in the MVP group. The 5-year survival rate in the MVP group was 89±6%. MVR was required in 1 patient 2 months after MVP because of increasing MR. Recurrence of endocarditis has not been observed in any case. CONCLUSIONS: Glutaraldehyde was safely used in a surgical intervention for AAIE in the mitral position with acceptable early and mid-term results.

Contrast-enhanced magnetic resonance imaging to detect chronic aortic dissection complicated by acute aortitis.

When chronic aortic dissection (CAD) is associated with aortic dilatation, the risk of aortic rupture increases. We report a case of CAD complicated by acute aortitis that was depicted in contrast-enhanced magnetic resonance imaging (MRI). Contrast-enhanced MRI allows early detection of subtle changes in the aortic wall as well as disease activity. Inflammation of aortic wall in the aortic dissection can be at higher risk of the dissected aortic expansion and rupture. When we recognize inflammation of unknown origin with CAD, contrast-enhanced MRI should be performed to rule out CAD complicated by acute aortitis may lead to catastrophic complications.

Choice of Aortic Valve Prosthesis in a Rapidly Aging and Long-Living Society.

PURPOSE: The aim of this study was to evaluate the long-term results of aortic valve replacement (AVR) with mechanical (M) and bioprosthetic (B) valves as recommended by the Japanese guidelines. METHODS: From April 1995 to March 2014, 366 adult patients underwent AVR. Of these, 127 (35%) patients received M and 239 patients (65%) received B valves. A retrospective analysis of the entire and the selected 124 patients aged 60 to 70 years was carried out. RESULTS: In patients aged 60 to 70 years, the 15-year survival and freedom from reoperation were 88% ± 7% and 100% for the M group and 34% ± 25% (p <0.001) and 73% ± 14% (p = 0.059) for the B group, respectively. Among propensity score matching of the subgroup, there was no significant difference in survival and freedom from reoperation. The rate of thromboembolism was higher in the M (M: 0.58% vs B: 0.35% patient per year, p <0.001) and the rate of hemorrhage was higher in the M group (M: 0.34% vs B: 0.12% patient per year, p <0.001). CONCLUSION: The current strategy of aortic valve choice based on the Japanese guidelines has provided excellent long-term results so far.

[Atrial Septal Defect with Hereditary Deficiency of Antithrombin III].

A 41-year-old female with hereditary deficiency of antithrombin III (ATIII) was diagnosed with atrial septal defect( ASD) and scheduled for the closure of ASD. She had been taking warfarin since she suffered from deep vein thrombosis 10 years ago. Preoperative management of anticoagulation included discontinuation of warfarin, and supplementation of antithrombin with heparin infusion. On the day of operation, antithrombin activity was maintained above 80% by administering antithrombin, and closure of ASD was carried out under standard cardiopulmonary bypass support using heparin. Heparin infusion was continued with antithrombin supplementation until prothrombin time-international normalized ratio(PT-INR) recovered to around 2.5 with warfarin. Her intra-and postoperative courses did not show any thromboembolic events, and she was discharged 20 days after the surgery.

Spontaneous closure of a large left ventricular pseudoaneurysm after mitral valve replacement.

Left ventricular pseudoaneurysm is a rare, but potentially fatal, condition that generally occurs as a complication of myocardial infarction, infective endocarditis, or cardiac surgery. Surgical repair is the treatment of first choice because of the marked risk of rupture, but deteriorated hemodynamics and complicated procedures to treat the pseudoaneurysm may lead to a high mortality rate. We report a 62-year-old woman with a large left ventricular pseudoaneurysm after mitral valve replacement for rheumatic mitral valve stenosis. Surgical repair was not performed due to the patient's refusal, but her pseudoaneurysm resolved spontaneously by 2 years after mitral valve replacement. Spontaneous obliteration of a large left ventricular pseudoaneurysm is very rare in a patient on warfarin therapy. This case suggests that a left ventricular pseudoaneurysm with a narrow neck may resolve spontaneously in rare settings.

Left ventricular pseudoaneurysm after reimplantation procedure.

Postoperative left ventricular pseudoaneurysm is a rare, but potentially lethal, complication because of the high risk of rupture and high mortality of repair. We report a 64-year-old man with Marfan syndrome who underwent the reimplantation valve-sparing aortic root replacement complicated by a postoperative left ventricular pseudoaneurysm that required urgent repair. Careful handling of the aortic root is required to avoid a left ventricular pseudoaneurysm, particularly in patients with connective tissue disorder.

Left ventricular pseudo-false aneurysm perforating into the right ventricle.

Left ventricular pseudo-false aneurysms are a very rare complication of myocardial infarction. Occasionally, they will perforate into the right ventricle. Their haemodynamic presentation is similar to postinfarction ventricular septal defect. Even with no early clinical symptoms, they can lead to congestive heart failure or sudden fatal rupture. We report on a 67-year old man who developed a large left ventricular pseudo-false aneurysm that perforated into the right ventricle late after acute inferior myocardial infarction. He had undergone percutaneous coronary intervention to the right coronary artery in August 2013. During the surgery performed in July 2014, with only incision at the aneurysm, the small perforation to the right ventricle was closed directly and the large perforation to the left ventricle was closed with a bovine pericardial patch. Considering the late mechanical complications attendant to left ventricular remodelling, physicians should conduct careful long-term follow-up of patients with transmural myocardial infarction, even if previous percutaneous coronary intervention was successfully performed.

Residual glycosaminoglycan accumulation in mitral and aortic valves of a patient with attenuated MPS I (Scheie syndrome) after 6 years of enzyme replacement therapy: Implications for early diagnosis and therapy.

Mucopolysaccharidosis (MPS) is an inherited metabolic disease caused by deficiency of the enzymes needed for glycosaminoglycan (GAG) degradation. MPS type I is caused by the deficiency of the lysosomal enzyme alpha-l-iduronidase and is classified into Hurler syndrome, Scheie syndrome, and Hurler-Scheie syndrome based on disease severity and onset. Cardiac complications such as left ventricular hypertrophy, cardiac valve disease, and coronary artery disease are often observed in MPS type I. Enzyme replacement therapy (ERT) has been available for MPS type I, but the efficacy of this treatment for cardiac valve disease is unknown. We report on a 56-year-old female patient with attenuated MPS I (Scheie syndrome) who developed aortic and mitral stenosis and coronary artery narrowing. The cardiac valve disease progressed despite ERT and she finally underwent double valve replacement and coronary artery bypass grafting. The pathology of the cardiac valves revealed GAG accumulation and lysosomal enlargement in both the mitral and aortic valves. Zebra body formation was also confirmed using electron microscopy. Our results suggest that ERT had limited efficacy in previously established cardiac valve disease. Early diagnosis and initiation of ERT is crucial to avoid further cardiac complications in MPS type I.

Distal aortic arch aneurysm associated with persistent fifth aortic arch.

Persistent fifth aortic arch (PFAA) associated with interruption of the fourth aortic arch is a rare malformation, usually requiring surgical intervention in childhood. A 51-year-old woman developed a distal aortic arch aneurysm associated with PFAA after patch aortoplasty for coarctation of the aorta at the age of 18. To our knowledge, this is the first reported case of a distal aortic arch aneurysm associated with PFAA. Residual tissues of the ductus and PFAA, which is not a definitive aortic arch, and the implanted synthetic patch may have been related to the development of the aneurysm. Graft interposition between the proximal border of PFAA and the descending aorta was performed because severe adhesions impeded the application of the fourth aortic arch as a proximal anastomotic site of the graft. Accurate diagnosis and sufficient understanding of PFAA allow an appropriate surgical treatment in childhood with a good result over the long term.