主动脉瓣四叶瓣畸形合并主动脉瓣病变的外科处理

目的 探讨主动脉瓣四叶瓣畸形对主动脉瓣功能的影响及其外科治疗.方法 2000年1月至2013年1月,我院通过经胸超声心动图和术中病理诊断主动脉瓣四叶瓣畸形13例.其中4例主动脉瓣的功能基本正常,9例合并主动脉瓣病变：重度关闭不全7例,重度关闭不全伴狭窄2例,合并二尖瓣中度关闭不全2例,三尖瓣中重度关闭不全1例.结果 13例患者中4例主动脉瓣功能正常者,继续随访中.9例合并主动脉瓣病变者,均行主动脉瓣置换术,同期行二尖瓣成形术2例、三尖瓣成形术1例.围术期无严重并发症及早期死亡,均康复出院.术后平均随访（6.34±5.17）年,心彩超提示主动脉瓣功能良好,无远期死亡.结论 主动脉瓣四叶瓣畸形是一种少见的先天性畸形,超声心动图有助于早期诊断.主动脉瓣功能正常时,可以随访;当合并主动脉瓣功能障碍时,应及时行主动脉瓣置换或修复,手术后可获得良好的远期效果.     

An Interesting Case of Tricuspid Aortic Valve with a Quadricuspid Function

Quadricuspid aortic valve (QAV) is a rare congenital heart defect, often related to severe aortic regurgitation, and usually detected by echocardiography or at the time of aortic valve surgery. We report a case of an interesting and extremely rare variant of “false” QAV, detected preoperatively by transthoracic and transesophageal echocardiography, in a severely symptomatic patient, admitted to our hospital for dyspnea. Three leaflets of aortic valve appeared quadricuspid, because the left coronary cusp was divided into 2 parts, as confirmed by MRI and pathology. Most frequently, QAV presents with all 4 leaflets equal in size.     

Quadricuspid aortic valves

Quadricuspid aortic valves (QAV) are a rare but well recognized cause of significant aortic regurgitation. The first case was found reported in 1862. Since then there have been 110 reported cases of QAV and we report 4 more. Previously, these were diagnosed at the time of surgery or postmortem examination. With advances in echocardiography, including harmonic imaging, and also the advent of transesophageal echocardiography, more cases are being diagnosed prior to surgery. We describe four more cases, three diagnosed preoperatively and one at the time of surgery, and then review the previously reported cases. Of the 114 cases reported, 46 had the aortic valve replaced, most commonly in the 5th and 6th decade of life. Hurwitz and Roberts classified quadricuspid valves according to the size of the leaflets. It has previously been believed that QAVs with four equal sized leaflets were less likely to develop significant aortic regurgitation; however, on review of the available cases, this would not appear to be the case. The preoperative diagnosis of QAVs is important as they can be associated with abnormally placed coronary ostium. Of the 114 cases reported, there are 10 reports of abnormally placed ostia. There has been at least one reported case of death occurring because of obstruction of an abnormally placed right coronary ostium by a prosthetic aortic valve.     

Long‐term performance of bicuspid and quadricuspid aortic valves: Similarities and differences

While bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, quadricuspid aortic valve (QAV) is rare. The usual three‐leaflet aortic valve is characterized by engineering advantages with superior long‐term performance, and thus, degenerative changes and significant functional deterioration appear at advanced age. Aim: Evaluation of long‐term performance, similarities, and differences between QAV and BAV. Methods: Screening of 19 000 consecutive echocardiographic studies was performed. Results: BAV was reported in 131 subjects with a prevalence of 0.7%, while QAV was seen in 11 with a prevalence of 0.06%, P < .00001. Age of BAV patients was younger, 45 ± 20 years vs 62 ± 17 years in QAV, P < .05, with higher proportion of females in those with QAV, 40% vs 30%. Chamber diameters were similar in both groups. Higher atrial contraction‐A‐wave mitral peak velocities and longer E‐wave deceleration times were found in subjects with QAV, P < .05 for both. Dilated ascending aorta was found in 25% of patients with BAV and in 18% of those with QAV, P = .2. Moderate and severe aortic valve stenosis were found in 21% of patients with BAV and in 27% of those with QAV, P = ns. More than moderate aortic regurgitation was found in 15.5% of BAV patients and in 9% of QAV, P = ns. Aortic valve infective endocarditis was found in 1.5% of BAV patients and in 9% of those with QAV. Conclusions: BAV is a common congenital anomaly, while QAV is rare. Similar prevalence of significant valve disease and aortopathy was found in both anomalies, though at younger age in BAV patients.     

Quadricuspid aortic valve with a hidden left ostium: Case report and literature review

Quadricuspid aortic valve (QAV) is a rare congenital condition that frequently progresses to aortic regurgitation with clinical impact in adulthood. Surgical treatment is required in the fifth to sixth decade of life in about one fifth of patients.We describe the case of a 64-year-old woman with regular cardiological follow-up for severe aortic valve regurgitation who had suffered recent clinical and echocardiographic deterioration. Conventional open surgery was indicated. During the procedure, a QAV with leaflet retraction and central orifice was observed. The aortic valve was successfully replaced.     

Echocardiographic and hemodynamic features of severe aortic regurgitation with diastolic opening of the aortic valve

Severe aortic regurgitation may be associated with premature aortic valve opening. Several possible etiologies for this diastolic opening have been suggested. We present a patient with hemodynamic data, M-mode and 2-D echocardiography in the setting of severe aortic regurgitation and diastolic aortic valve opening. Our data lead us to conclude that aortic valve opening in this situation is neither from passive flotation nor dependent on atrial systole. We believe that active ventricular recoil mechanisms can facilitate increases in diastolic ventricular pressure which then can transiently exceed aortic pressure in the setting of severe aortic regurgitation. This hemodynamic observation suggests that the valve opening is an active process.     

Retrograde percutaneous aortic valve implantation for critical aortic stenosis.

Recently, a prosthetic aortic valve has been implanted percutaneously in several patients using an antegrade transseptal approach. This has been shown to be feasible and associated with dramatic hemodynamic improvement. We report a retrograde implantation of a percutaneous heart valve (PHV) in an 84-year-old man with critical aortic stenosis and refractory congestive heart failure after difficulties encountered with an initial antegrade approach. While attempting antegrade transseptal implantation of a PHV, the anterior leaflet of the mitral valve was tethered by the guidewire resulting in severe mitral regurgitation and pulseless electrical activity. Cardiac resuscitation was successful. Utilizing a retrograde approach, the PHV was successfully implanted in a stable position below the coronary ostia and well above the mitral valve leaflets. The aortic valve area increased from 0.55 to 1.7 cm2 with only mild paravalvular aortic regurgitation. Despite marked improvement in aortic valve function, the patient died secondary to guidewire-induced mitral valve anterior leaflet laceration, severe mitral regurgitation, and cardiogenic shock. Retrograde implantation of a PHV can be successfully performed with substantial increase in aortic valve area and an acceptable degree of aortic regurgitation. Although the retrograde approach may be associated with greater risk of vascular access site complications, it may be considerably safer by avoiding potential guidewire injury to the mitral valve. Further refinements in technique may establish the retrograde approach as the preferred means of PHV implantation in nonsurgical patients with critical aortic stenosis.     

A case of 75-year-old survivor of unrepaired tetralogy of Fallot and quadricuspid aortic valve

In tetralogy of Fallot (TOF), the most common form of cyanoticcongenital heart disease, only a few patients reach adulthoodwithout surgical correction. We present the case of a man withTOF who survived until the age of 75 years without surgicalintervention and had a very unusual combination of TOF and quadricuspidaortic valve (QAV). QAV, complicated by aortic regurgitation,is an uncommon finding in TOF patients. The hemodynamic consequencesfor both the right and left ventricles are significant. Thiscase provides a rare insight into the late outcome of an elderlypatient with uncorrected TOF.     

Severe intraprosthetic regurgitation and valve embolization after transcatheter aortic valve implantation

Transcatheter aortic valve implantation is a novel therapeutic approach for high‐risk patients with severe symptomatic aortic stenosis. The success rate of this new procedure is high; however, procedural complications can occur and may result in devastating outcomes. Here, we report a case of transfemoral catheter aortic valve implantation using the Edwards SAPIEN valve complicated by severe intravalvular leak due to immobile cusp associated with shock. We treated with a second valve that embolized and deployed in the descending aorta. A third valve was then deployed within the first with elimination of aortic regurgitation and immediate hemodynamic improvement. © 2012 Wiley Periodicals, Inc.     

Transfemoral transcatheter aortic valve implantation after aortic valve repair with HAART 300 device

We report the first successful case, to our knowledge, of CoreValve Evolut R (Medtronic, Minneapolis, MN) implantation into a failed HAART 300 aortic annuloplasty device (BioStable Science & Engineering, TX). An 81‐year‐old man presented with severe symptomatic aortic regurgitation secondary to failure of the 21 mm HAART 300 device, which had been implanted 45 days previously. Transthoracic echocardiography (TTE) revealed grade 3 aortic regurgitation with central jet, without aortic valve stenosis. Because of the high risk for redo surgery, the heart team proceeded with femoral transcatheter aortic valve implantation. The 26 mm CoreValve Evolut R was deployed into the 21 mm HAART 300 device without difficulty or complications. There were no intraoperative or postoperative complications. The patient was discharged after 5 days. TTE showed a mean aortic valve gradient of 18 mmHg, with minimal paravalvular leak. Our experience suggests that CoreValve Evolut R implantation may be an attractive option in patients with failed HAART 300 aortic annuloplasty.     

Type A quadricuspid aortic valve and ascending aorta aneurysm: a rare combination

Quadricuspid aortic valve (QAV) is a rare congenital anomaly associated with aortic valve insufficiency and significant morbidity, and requires the replacement or, rarely, the repair of the malfunctioning heart valve. A QAV associated with an ascending aorta aneurysm is an extremely rare anatomic combination with a hypothetical, but not clear, shared embryological etiology. To date, only two cases of type B QAV with ascending aorta aneurysm have been reported. Herein is described the first ever case of a 38-year-old male suffering from severe symptomatic aortic valve regurgitation due to a type A QAV, associated with an ascending aorta aneurism, who underwent a successful combined replacement of the aortic valve and ascending aorta.     

Salvaging lowermost deployment of an acurate device during transcatheter aortic valve replacement with balloon and lasso pull techniques

Transcatheter aortic valve replacement (TAVR), also known as transcatheter aortic valve implantation (TAVI), is being used with increasing frequency in patients with severe aortic stenosis at high or prohibitive surgical risk. A number of devices are becoming available for TAVR, and competence in using them is mandatory to maximize the safety and efficacy of TAVR, while individualizing device selection in keeping with patient features. The ACURATE TF is a novel promising device for transfemoral TAVR. However, its peculiar features may require additional maneuvers in case of complications. We hereby report the case of a patient undergoing transfemoral TAVR with the ACURATE TF device, in whom lowermost deployment was complicated by massive aortic regurgitation. With two separate remedial actions, the balloon pull and lasso techniques, we were able to pull back the device and significantly reduce post‐TAVR aortic regurgitation. Awareness of this complication and the possible use of these two techniques may increase the safety and efficacy of TAVR with this and other new devices. © 2015 Wiley Periodicals, Inc.     

Transcatheter aortic valve implantation for severe pure aortic regurgitation due to active aortitis

Aortitis is an uncommon systemic inflammatory disease affecting the aorta and its main branches. Severe aortic regurgitation (AR) represents a fearsome complication of aortitis and is associated with an increased mortality rate. Surgical aortic valve replacement represents the only treatment choice for these patients. However, it is associated with a higher risk of medium to long-term complications such as prosthetic valve detachment. This is the first reported case where severe AR secondary to aortitis was managed with transcatheter aortic valve implantation (TAVI). TAVI was safe and effective in this clinical setting and may be considered a viable alternative to high-risk surgery in these complex patients.     

A quadricuspid aortic valve with atrial septal defect

A quadricuspid aortic valve (QAV) is a rare congenital heart defect, previously described as an incidental finding at the time of surgery or postmortem, which is now being increasingly detected by 2D transthoracic or transesophageal echocardiogram. With advances in echocardiography, secondary cardiac anomalies are also being described in association with QAV. Herein we describe a patient with QAV with a secundum atrial septal defect.     

A case of prosthetic aortic valve dehiscence due to infective endocarditis without paravalvular regurgitation

Aortic prosthetic valve endocarditis is often a challenging disease process that carries high morbidity and mortality. Echocardiography is widely used to identify infected valves and associated complications. One major complication of an infection involving the aortic annulus is dehiscence of the prosthetic valve from the aortic root and is usually associated with paravalvular regurgitation. Here, we present a rare case of complete prosthetic valve dehiscence without paravalvular regurgitation on transthoracic and transesophageal echocardiography.     

Quadricuspid Aortic Valve: A Case Report and Review of the Literature

The quadricuspid aortic valve (QAV) is a rare malformation; often isolated, sometimes associated with other heart diseases. Before the era of echocardiography, the diagnosis was made incidentally at autopsy or during surgery of valve replacement. The extensive use of echocardiography has allowed an early and accurate diagnosis of this malformation. In many cases, the transthoracic approach is suitable for the diagnosis but, transesophageal echocardiography is a tool for the accurate definition of the valve anatomy. This review analyzes, after the presentation of a clinical case, the current knowledge on embryogenesis, classification, diagnosis and clinical course of QAV.     

Complications after Transfemoral Transcatheter Aortic Valve Replacement with a Balloon‐Expandable Prosthesis: The Importance of Preventative Measures and Contingency Planning

Transcatheter aortic valve replacement (TAVR) with balloon‐expandable Edwards‐SAPIEN valve was superior to standard therapy in inoperable patients and noninferior to surgical aortic valve replacement in high surgical‐risk, but operable patients, with severe symptomatic aortic stenosis in the randomized controlled PARTNER trial. Since the first case of TAVR with a balloon‐expandable valve in 2002, several groups have reported their experience with balloon‐expandable valves with high‐procedural success. In the United States, the balloon‐expandable Edwards‐SAPIEN valve is the only transcatheter heart valve approved by the FDA for commercial use. Moreover, this is only in high‐risk inoperable patients. Despite increasing experience with the TAVR procedure, it can be associated with complications, which can be technically challenging, even for an experienced operator. Complications associated with TAVR include vascular complications, valve malpositioning, regurgitation, embolization, coronary compromise, conduction abnormalities, stroke/transient ischemic attack, acute kidney injury, cardiac tamponade, and hemodynamic collapse. A thorough understanding of the procedure is essential for pre‐emptive planning for procedural complications and early identification and management of complications are necessary for procedural success. We hereby review our experience of transfemoral TAVR with balloon‐expandable valves, offer practical tips to maximize the likelihood of procedural success, describe pre‐emptive strategies to prevent peri‐procedural complications and bailout measures to manage them, should they occur. © 2018 Wiley Periodicals, Inc.     

Aortic root complications of infective endocarditis--influence on surgical outcome

Fifty cases of aortic valve endocarditis during a 6-year period between 1982 and 1988 were reviewed. Twenty-three (46%) had aortic root complications by way of aortic root abscess or mycotic aneurysm in the perivalvular area. Patients with root complications were grouped into the aortic root abscess (ARA) group and those without into a non root abscess (NARA) group. Prosthetic valve endocarditis dominated in the ARA group (12 and four cases of prosthetic valve infection in the ARA and NARA groups, respectively; P less than 0.01). Surgical mortality was significantly higher at 13.6% in the ARA group as opposed to 2.2% in the NARA group (P less than 0.05). Post-operative aortic regurgitation was present in 8 (57%) of 14 patients in the ARA group surviving surgery but in only two (8.7%) of 23 patients in the NARA group (P less than 0.03). We conclude that aortic root complications are a frequent occurrence in aortic valve endocarditis, lead to an increased operative mortality and is associated with a high incidence of post-operative aortic regurgitation.     

Mixed aortic valve disease treated with transcatheter aortic valve replacement in a high risk patient presenting with acute decompensated heart failure

Mixed aortic valve disease refers to the combination of aortic regurgitation (AR) and aortic stenosis (AS). Commonly etiologies include a bicuspid aortic valve, rheumatic heart disease, and endocarditis superimposed upon a stenotic aortic valve. Treatment depends upon the severity of disease, the presence of symptoms and the size and function of the left ventricle. We present a case of a young patient that presented with new onset acute decompensated heart failure with mixed aortic valve disease that was successful treated with transcatheter aortic valve replacement (TAVR). Invasive hemodynamics at baseline and following TAVR provide an insight into the characteristic features of mixed aortic valve disease. TAVR represents a new treatment option for critically ill patients deemed high risk or nonoperable for surgical aortic valve replacement.     

Quadricuspid aortic valve: report of three cases

Quadricuspid aortic valve (QAV) is a very rare congenital malformation. We have encountered three patients with QAV, of whom one patient may be the eldest reported patient with this particular anatomical abnormality. In another of our patients, there was aortic regurgitation, aortic stenosis, and healed infective endocarditis, with adhesion of the tips of the cusps. In all three patients, the cusps were all of equal size. Until now, there has been very little documented evidence about the anatomical variations in QAV or its relationship with infective endocarditis. From the available literature, we conclude that the anatomical variations in patients with QAV are similar to those in patients with quadricuspid pulmonary valve, and infective endocarditis may not be an uncommon complication.