Quadruple valve involvement in a patient with severe carcinoid heart disease

We report on the case of a 48-year-old male patient suffering from a severe carcinoid heart disease with involvement of the four valves. The primary carcinoid tumor was diagnosed in the ileum. The patient developed a moderate stenosis and severe insufficiency of the tricuspid valve, a severe insufficiency of the pulmonary valve, and a moderate insufficiency of the mitral and aortic valves. Ultimately, a stentless pulmonary valve replacement was performed, as well as a tricuspid valve replacement with a pericardial prosthesis and aortic and mitral valve plasty. The patient recovered well and he was discharged from hospital on day 10.     

Right- and left-sided carcinoid heart disease without intracardiac shunting

We present a case of a 54-year-old man with carcinoid heart disease and mitral valve involvement. He had hepatic carcinoid with an extremely elevated urinary excretion of 5-hydroxyindole acetic acid and was referred to our division for shortness of breath and leg edema. Transthoracic echocardiography showed the thickened and retracted tricuspid valve leaflets and severe tricuspid regurgitation. Moderate mitral regurgitation was observed, with the involvement of mitral valve leaflets. A patent foramen ovale was not detected on transesophageal echocardiography. The extremely high concentration of circulating serotonin and/or other vasoactive substances may have contributed to left- as well as right-sided carcinoid heart disease.     

An evaluation of cases carried out by both root reconstruction using carrel patch method and concomitant procedure

During the past 7 years from January 1991 to November 1997, we experienced 31 cases of aortic root reconstruction utilizing Carrel patch method. Concomitant procedure were performed in 9 cases of them due to another cardiovascular disease. Complicated cardiovascular disease included 3 cases of ischemic heart disease, 3 cases mitral regurgitation and one case of Aortic arch aneurysm. Several concomitant procedures were performed; 5 cases of CABG, 2 cases of mitral annuloplasty, one case of CABG with mitral valve replacement and one case of aortic arch replacement. The mean extra corporeal circulation time was 190.6 ± 39.3 minutes and aortic clamp time was 147.8 ± 34.2 minutes in these 9 cases. There were no significant differences of operative results between the simple root reconstruction group and the concomitant procedure group. We concluded that the aortic root reconstruction using the Carrel patch method has few complications. Although further long-term follow-up is required, our experiences suggest that the aortic root reconstruction with the concomitant procedure can be carry out safely with the aid of appropriate assistance method.     

A study of the subendocardial ultrastructural changes in the volume-overloaded left ventricular hypertrophy: A comparison of the effects of aortic and mitral regurgitation

Ultrastructural changes of the subendocardial layer of the volume-overloaded left ventricle were studied in patients who underwent open heart surgery for chronic aortic valve disease (4 patients) and mitral valve disease (6 patients). Patients in these two entities were estimated to be in the same level of hemodynamic functional capacity. In aortic valve disease, the subendocardial layer of the hypertrophied left ventricle showed degeneration of cardiac muscle cells. Conversely, degeneration of cardiac muscle was rarely observed in those with mitral valve disease. Numerous small and medium sized mitochondria were observed in the subendocardial layer of the left ventricle in aortic valve and mitral valve diseases and suggested that the patients in both groups were well compensated at the time of surgery. Moderate to severe dilatation of tubules of the sarcoplasmic reticulum was observed with significantly higher incidence in the case of aortic valve disease. The hypertrophied myocardium as a result of aortic valve disease showed more ischemic damage than that due to mitral regurgitation, even with the same levels of hemodynamic functional capacity. These data suggest that the myocardium in cases of mitral regurgitation tolerates open heart surgery to a much greater extent and that the prognosis is better.     

An experience of a case with perforation of an aneurysm in the anterior mitral cusp developing after aortic valve replacement.

Mitral valve aneurysm is a rare disease and in Japan, cases of perforation which is considered to be mainly caused by infectious endocarditis is usually only encountered through case reports. We experienced a case who received aortic valve replacement and mitral valve annuloplasty for combined valvular heart disease of aortic insufficiency and mitral insufficiency followed by mitral valve replacement for severe mitral valve regurgitation subsequent to perforation of the anterior mitral cusp, leading to recovery.     

Influence of concomitant mitral valve dysfunction on survival after aortic valve replacement

Aortic valve pathology is the most common acquired valvular heart disease in the adults of western countries, and mitral regurgitation (MR) is often clinically present in patients with degenerative aortic stenosis or insufficiency. Many studies report an incidence of MR between 65-75% in patients evaluated for aortic valve replacement. Severe aortic valve disease may be associated with functional mitral regurgitation (FMR) defined as the failure of mitral valve to prevent systolic backward flow in the absence of any significant structural or intrinsic valvular disease. Increased afterload and left ventricular remodeling have been implicated to explain FMR in patients with aortic valve disease. Moreover, organic mitral valve disease can be associated with aortic stenosis and can be rheumatic or degenerative. We have examined the data of the literature to understand the evolution of MR, the impact of mitral regurgitation on the outcome of patients undergoing aortic valve replacement, and to determine clinical predictors of prognosis in patients with concomitant MR at the time of aortic valve replacement.     

Leakage test during mitral valve repair

Mitral valve repair is the preferred surgical treatment for mitral regurgitation. Cardiac surgeons must increasingly pursue high-quality mitral valve repair, which ensures excellent long-term outcomes. Intraoperative assessment of a competency of the repaired mitral valve before closure of the atrium is an important step in accomplishing successful mitral valve repair. Saline test is the most simple and popular method to evaluate the repaired valve. In addition, an “Ink test” can provide confirmation of the surface of coaptation, which is often insufficient in the assessment of saline test. There are sometimes differences between the findings of the leakage test in an arrested heart and the echocardiographic findings after surgery. Assessment of the mitral valve in an arrested heart may not accurately reflect its function in a contractile heart. Assessment of the valve on the beating heart induced by antegrade or retrograde coronary artery perfusion can provide a more physiological assessment of the repaired valve. Perfusion techniques during beating heart surgery mainly include antegrade coronary artery perfusion without aortic cross-clamping, and retrograde coronary artery perfusion via the coronary sinus with aortic cross-clamping. It is the most important point for the former approach to avoid air embolism with such precaution as CO₂ insufflation, left ventricular venting, and transesophageal echocardiography, and for the latter approach to maintain high perfusion flow rate of coronary sinus and adequate venting. Leakage test during mitral valve repair increasingly takes an important role in successful mitral valve reconstruction.     

Reoperations in valvular heart disease

Background Reoperations for valvular heart disease are associated with a higher overall mortality than the primary operations. In this retrospective analysis, we present our experience of reoperative valvular heart surgery over a period of 25 years. Methods From January 1975 to July 2000, 13039 operations were performed for valvular heart disease. Of these 665 were reoperations. The mean age of the patients at the primary operation was 24.0±10.2 years (range: 8 to 65 years) and at re-operation was 35.6±11.6 years (range: 9 to 65 years) with an interval of 9.4±2.2 years (range: 0.2 to 25 years) between the 2 procedures. Four hundred and forty reoperations were performed following a previous closed mitral valvotomy and procedures included, redo closed mitral valvotomy (n=28), mitral valve replacement (n=30), open mitral commissurotomy (n=51), mitral valve repair (n=9), homograft mitral valve replacement (n=2), double valve replacement (n=47), aortic valve replacement (n=2) and homograft aortic valve replacement plus open mitral commissurotomy (n=l). Eighty six patients underwent reoperations following mitral valve replacement. Valve thrombosis (n=50) and endocarditis (n=10) were principle causes of reoperation. Forty three patients required reoperation following failed mitral valve repair, 19 following open mitral commissurotomy and 8 following homograft mitral valve replacement. Sixty five patients underwent reoperation following aortic valve operations: prosthetic aortic valve replacement in 43, homograft aortic valve replacement in 5, aortic valve repair in 10, and Ross procedure in 7. Results Majority of patients were operated through midsternotomy. Aortic cannulation was possible in all but 4 patients in whom femoral artery cannulation was required. Operative mortality following reoperations was 7.5% (n=50). Peri-operative bleeding, low cardiac output and infective endocarditis were major causes of operative deaths. Other post-operative complications included cerebrovascular accident (n=3), acute renal failure (n=10) and jaundice (n=25). Fifteen patients developed significant wound infection. Conclusions Patients undergoing operation for valvular heart disease frequently require reoperation. Reoperative valvular heart surgery is safe and can be undertaken with acceptable mortality and morbidity.     

Current surgical treatment for acquired valvular heart disease

We discuss the current status of surgical treatment for acquired valvular heart disease. Mitral valve repair for organic and functional mitral regurgitation is the first choice instead of valve replacement. It is important that surgery for functional mitral regurgitation restores the geometry of the left ventricle and mitral valve. The reduction of mitral valve tethering for functional mitral regurgitation is a current topic of discussion. At present, the surgical procedure for both aortic stenosis and aortic regurgitation is valve replacement in most cases, although aortic valve repair has been attempted for aortic regurgitation in recent years. The early results of aortic valve repair are excellent, but the long-term results have not been clarified. The durability of valve repair in both the mitral and aortic position is a future issue and it may be improved by revising the indications for valve repair and using new surgical techniques.     

重症心脏瓣膜病的外科治疗

目的总结重症心脏瓣膜病的外科治疗结果,探讨提高早期生存率的措施。方法自2000年6月至2005年7月,对78例重症心脏瓣膜病患者施行瓣膜替换术。其中单纯二尖瓣置换12例,二尖瓣置换 三尖瓣成形22例,单纯主动脉瓣置换8例,二尖瓣 主动脉瓣置换 三尖瓣成形35例,二尖瓣置换 冠状动脉旁路移植术1例。结果死亡6例,其中术后并发低心排血量5例,心室颤动治疗无效死亡1例,死亡率7.69%。随访53例,平均随访2.5年,死亡5例。结论对重症心脏瓣膜病患者,注重改善术前心功能,掌握手术时机,尽量保留瓣下组织,选择合适瓣膜,重视围手术期处理,可提高手术成功率。     

Combined techniques for double valve replacement in the infant

A 6-month-old female infant was seen with heart failure secondary to severe aortic and mitral regurgitation. As a neonate the infant had undergone an aortic valvotomy for congenital aortic stenosis. Subsequently the infant had aortic and mitral regurgitation with an infarcted papillary muscle. Double valve replacement was carried out with the St. Jude valve. The first approach was by the Manouguian procedure with extension of the aortotomy out between the left coronary cusp and the noncoronary cusp. The posterior mitral apparatus was resected, and a 19-mm St. Jude aortic valve was sewn into the mitral position. Because the enlarged aortic valve annulus was still inadequate to accommodate a 19-mm St. Jude valve, a Konno procedure was carried out to enlarge the aortic ring anteriorly. Atrial, septal, and aortic repair and right ventricular outflow tract reconstruction were carried out with bovine pericardium. Bypass was carried out with standard techniques of hypothermia, aortic cross-clamping, and cardioplegia. Postoperative anticoagulation therapy was initially with aspirin and dipyridamole (Persantine); however, clotting of the mitral prosthesis necessitated treatment with urokinase and heparin, which completely resolved the clot. Sodium warfarin (Coumadin) therapy was then begun. One year postoperatively, the child is developing normally.     

Techniken der minimal-invasiven Mitralklappenchirurgie

Mitral valve disorders are the second most common heart valve disease in Europe and other industrialized nations. In mitral reconstruction the gold standard therapy is mitral valve repair. Minimally invasive techniques have been developed since the 1990s. As a first step techniques were introduced to establish a cardiopulmonary bypass through alternative access routes, namely the inguinal blood vessels. For aortic clamping two techniques are available: endoaortic balloon and transthoracic cross clamping. Besides video-assisted surgery by a right lateral minithoracotomy and direct vision, totally endoscopic and robot-assisted surgery is also available. For all techniques excellent operative results as well as good short and long-term outcomes have been described.     

Klappenchirurgie

Indications for surgery form part of the guidelines for the treatment of valvular heart disease. The goal is to avoid risk associated with surgery as long as possible without reaching the point of irreversible damage to the heart and other organs. Since improvements of surgical techniques have reduced the operative risk these guidelines need revision periodically. Treatment of symptomatic patients is mainly consented. Despite this consensus surgery is denied to a significant number of patients with symptomatic aortic stenosis. Treatment of patients with severe but asymtomatic valvular heart disease is under debate. Excercise testing to detect surgical candidates is recommended but rarely performed. Evidence is evolving that subgroups of patients with asymptomatic aortic stenosis benefit from early surgery, but identification of these patients remains difficult. Advances in surgical reconstruction of leaking mitral or aortic valves have led to recommendations for earlier surgery of mitral insufficiencies; data generation for reconstructive surgery of the aortic valve is underway. Surgical experience with these techniques is of major importance in the process of deciding when to operate.     

Triple valve replacement in a patient with severe carcinoid heart disease

We report on the case of a 34-year-old male patient suffering from end-stage carcinoid heart disease with severe tricuspid, pulmonary and mitral valve regurgitation. In addition, a persisting foramen ovale was present. The primary carcinoid tumor was never discovered. However, urine 5-hydroxy-indole-acetic-acid (5-HIAA) were consistently elevated after the first diagnosis of carcinoid disease and after eight years of medication with Octreotide and Interferon alpha-2b our patient developed significant cardiac insufficiency mainly due to severe valvular dysfunction. Ultimately, mechanical tricuspid, mitral and pulmonary valve replacement was performed. Twelve hours following the operation the patient had to be returned to the operating room for persisting intrathoracic hemorrhage. He recovered uneventfully and was discharged from hospital on day 37. Twelve months following triple valve replacement the cardiac status recovered from preoperative NYHA-IV to NYHA-I.     

Zur Leitlinie Herzklappenerkrankungen der ESC und EACTS Version 2012

Heart valve disease is the most common disease of the cardiovascular system besides coronary heart disease. After aortic valve stenosis, mitral valve and tricuspid valve diseases also have a high incidence. Surgical intervention is currently the gold standard therapy. Perioperative and long-term results are excellent. An increase in the quality of life can be durably achieved for most patients. New therapies for high-risk patients are available that can be used to treat mitral valve disease without the use of a cardiopulmonary bypass. MitraClip procedures for the treatment of severe mitral valve insufficiency show good short-term results as well but comprehensive long-term results are lacking. This second part of the article summarizes the evidence and recommendations for the treatment of acquired diseases of mitral and tricuspid valves (except endocarditis) from the recent guidelines on the management of valvular heart disease which were published for the first time by the European Society of Cardiology (ESC) in conjunction with the European Association of Cardio-Thoracic Surgery. In the first part the recommendations for the treatment of aortic valve diseases were summarized.     

Combined Procedure of Coronary Artery Bypass Grafting and Valve Repair

The safety of combined operative procedures for valvular and coronary artery disease was reviewed in 27 patients. Twelve patients had aortic valve disease and 15 had mitral valve disease. Forty-seven coronary artery reconstructions were performed, an average of 1.7 per patient. Twenty-two patients underwent valve replacement and 5 had valvuloplasty. Congestive heart failure was the major symptom in 20 patients, and angina was the major symptom in 7. Eight of the patients with congestive heart failure had no angina, but significant coronary stenoses were demonstrated at routine coronary angiography.Coronary reconstruction was performed before valve repair. Two patients died postoperatively (a hospital mortality of 7.4%), and there were 4 late deaths from 2 to 28 months postoperatively. There were no postoperative myocardial infarctions.Contrary to previous reports, coronary artery reconstruction and valve repair need not be associated with an increased risk. Protection of the myocardium by coronary perfusion through reconstructed coronary arteries enables valve repair to be done without greater risk than valve repair alone. All patients considered for valve repair should have coronary angiography.     

A rapid structural degeneration of a porcine mitral valve

A 73-year-old woman underwent both mitral and aortic valve replacements with porcine heart valve prostheses because of severe mitral regurgitation and severe aortic regurgitation. Ten months after surgery, maximal flow velocity of the aortic valve reached 4.6 m/sec and moderate mitral regurgitation was detected. Repeated mitral and aortic valve replacements with mechanical heart valves were performed. The excised mitral valve showed thinning of the 3 cusps, and 2 of them were perforated. There was pannus overgrowth on the flow surface of the porcine aortic valve. Histologic examination of the excised mitral valve revealed marked inflammatory changes with macrophages.     

Mitral valve‐in‐valve replacement supported by a transapically snared guidewire via septostomy

We describe a 72‐year‐old woman, a known case of rheumatic heart disease with a history of mitral and aortic valve replacement 8 years previously, who underwent mitral valve‐in‐valve replacement supported by a transapically snared guidewire through septostomy.     

先天性心脏病术中心脏瓣膜的保护和矫治

目的 总结先天性心脏病术后因瓣膜功能不全再手术的经验。方法 回顾分析先天性心脏病术后再行瓣膜手术13例患者的临床资料,其中室间隔缺损修补术后8例,部分心内膜垫缺损修补术后3例,法洛四联症和房间隔缺损修补术后各1例。第1次手术时即存在二尖瓣轻～中度关闭不全6例,主动脉瓣关闭不全1例;新出现瓣膜功能异常6例,其中2例因补片漏致三尖瓣关闭不全,2例因前叶腱索断裂致三尖瓣关闭不全,1例因残留右心室流出道狭窄继发三尖瓣关闭不全,1例因伤及主动脉瓣并发二尖瓣和三尖瓣关闭不全。13例中,行二尖瓣置换6例,三尖瓣置换2例,主动脉瓣置换1例,行主动脉瓣置换并二尖瓣、三尖瓣成形1例,三尖瓣成形3例。同时修补残余漏,疏通右心室流出道。结果 术后发生低心排综合征3例。2例术后早期分别死于脑气栓和呼吸循环衰竭。11例术后痊愈出院,随访1～8年,心功能良好。结论 先天性心脏病矫治术中应注意心脏瓣膜的保护,合并的瓣膜功能异常应积极修补,及时地再手术可取得良好效果。     

A case report of aortic and mitral regurgitation combined with ankylosing spondylosis

The purpose of this communication is to present a successful case of surgical treatment of aortic and mitral valve regurgitation associated with ankylosing spondylosis. A 48-year-old male was admitted with symptoms of palpitation and shortness of breath. His heart murmur had been pointed out for 4 months prior to this admission. His previous history did not show any evidence of rheumatic heart disease. Retrograde aortography showed moderate to severe aortic valvular regurgitation and echocardiography revealed mitral valve regurgitation with minor degree. At the same time he complained of rigidity of neck muscle as well as back pain for last 15 years. He underwent aortic valve replacement and mitral valve was left alone. His postoperative course was uneventful. In Japan, aortic valve regurgitation associated with ankylosing spondylosis has been reported to be very rare compared to the European or American people. Several important features in the diagnosis and operative treatment for this combination of diseases have been also discussed.