Echocardiographic findings of cardiovascular involvement in Behçet's disease and post-operative complications after cardiac surgery.

OBJECTIVES: The aim of this study was to analyse the spectrum of echocardiographic findings in patients with cardiovascular involvement in Behçet's disease (BD) and followed up the post-operative complications. METHODS: We enrolled 26 BD patients who underwent first cardiac surgery in Anzhen Hospital, Beijing, China. Medical records and echocardiographic findings were retrospectively analysed. RESULTS: The 26 patients consisted of 4 women and 22 men. 22 (84.6%) of the patients were diagnosed with moderate/severe aortic regurgitation (AR). Some distinctive echocardiographic features with AR were observed, including prolapse of aortic cusps, vegetation-like mobile lesions, an echo-free space mimicking aortic root abscess and aortic aneurysm formation. 3 (11.5%) of the patients were diagnosed with isolated descending aortic aneurysm. 1(3.8%) of the patients was diagnosed with pulmonary artery aneurysm. BD was preoperatively diagnosed by clinicians in 20 patients. And 6 patients were diagnosed post-operatively by clinicians. In a total of 26 patients, post-operative complications occurred in 8 (30.7%) patients. The complications occurred in the 6 patients diagnosed post-operatively and 2 patients diagnosed pre-operatively. The post-operative complications of these patientsincluded aortic paravalvular leakage, coronary-graft anastmotic leakage and mitral paravalvular leakage. CONCLUSIONS: The most common echocardiographic feature of cardiovascular involvement in BD is severe aortic regurgitation with prolapse of aortic cusps, vegetation-like mobile lesions, an echo-free space mimicking aortic root abscess or aortic aneurysm formation. Accurate preoperative diagnosis of BD is beneficial to the choice of immunosuppressive therapy before and after surgery, which is likely to reduce postoperative complications especially for patients with severe lesions.

Long-Term Results after Aortic Valve-Sparing-Reimplantation Operation (David) in Bicuspid Aortic Valve.

BACKGROUND AND AIM OF THE STUDY: The aortic valve-sparing reimplantation operation (David) is increasingly used in patients with aortic root aneurysm and intact cusps. David's procedure is also feasible in patients with bicuspid aortic valve (BA), though few long-term data are available. METHODS: An analysis was conducted of the long-term echocardiographic data from patients with BAV who had undergone David's procedure at early and long-term follow up (FU) examinations. RESULTS: Between 1994 and 2010, a total of 30 patients with BAV underwent David's procedure at the authors'institution. There were no in-hospital or late deaths, and the mean long-term FU was 6.64 ± 3.54 years (range: 3.2-20.1 years). The mean aortic regurgitation (AR) grade was increased from 0.26 ± 0.37 at early FU to 0.70 ± 0.80 at long-term FU (p = 0.013). Four patients (13%) had to be reoperated after 10.00 ± 4.74 years (range: 5.49-17.06 years) due to aortic stenosis (n = 1) and aortic insufficiency (n = 3); the latter three patients had a significant prolapse of both the fused and the non-coronary cusp of reconstruction. CONCLUSION: The mean AR grade was increased significantly but was ≤ I-II in 59% of patients and ≤ II in 93% of patients after a mean FU of 6.6 years. Patients with prolapsing non-coronary leaflet at reconstruction seemed vulnerable to recurrent AR. Among patients, survival was excellent, and the reoperation rate and hemodynamics acceptable. Long-term follow up data are necessary to further refine the surgical techniques employed and to improve the results achieved.

Successful percutaneous management of partial avulsion of the native aortic valve complex complicating transcatheter aortic valve replacement.

This is the first reported case of partial aortic valve and annulus rupture during transcatheter aortic valve replacement, and its successful management through percutaneous means. It stresses the fact that even severe procedural complications can often be treated by a heart team endovascularly, without requiring sternotomy.

Valvular heart disease in patients with osteogenesis imperfecta.

Osteogenesis imperfecta (OI) or "brittle bone disease" is a rare connective tissue hereditary disorder. The most common clinical presentation of OI is bone fractures. OI also involves extraskeletal structures; however, cardiovascular manifestations are rare. In this report, we describe the cardiovascular anomalies of patients with OI who underwent valve surgery and review the literature on this subject.

Aorta-to-right ventricular outflow tract fistula with coronary cusp prolapse.

We describe a rare case of aorta-to-right ventricular fistula in a 20-year-old man who presented with signs and symptoms of congestive heart failure. He was successfully treated by closing the fistula with a Dacron patch from the aortic side. At follow-up, he was asymptomatic with no left-to-right shunt.

Supracristal ventricular septal defect with severe right coronary cusp prolapse.

The case of a 20 years old male, diagnosed as supracristal ventricular septal defect (VSD) for last 6 years is being presented. He came in emergency department with decompensated congestive cardiac failure. After initial stabilization, he underwent transthoracic echocardiogram which showed large supracristal VSD, severely prolapsing right coronary cusp, severe aortic regurgitation and severe pulmonary hypertension. Right heart catheterization was performed which documented reversible pulmonary vascular resistance after high flow oxygen inhalation. He underwent VSD repair, right coronary cusp was excised and aortic valve was replaced by mechanical prosthesis. Post operative recovery was uneventful. He was discharged home in one week.

Aortic regurgitation caused by rupture of the abnormal fibrous band between the aortic valve and aortic wall.

This report documents the sudden onset of aortic regurgitation (AR) by an exceptional cause. A 68-year-old woman suddenly experienced general fatigue, and AR was diagnosed. One year later, we performed aortic valve replacement. At surgery, three aortic cusps with a larger noncoronary cusp had prolapsed along with a free-floating fibrous band that had previously anchored the cusp to the aortic wall. Its rupture had induced the sudden onset of AR. There was no sign of infectious endocarditis. We performed successful aortic valve replacement.

Ventricular septal defect and associated complications.

OBJECTIVE: To determine the frequency of various types of ventricular septal defects (VSD) and associated complications in local paediatric population. METHODS: A cross sectional descriptive study was conducted on children undergoing echocardiography in a single centre from January 2006 to December 2009 at Paediatric Cardiology Department, Ch. Pervaiz Elahi Institute of Cardiology Multan- Tertiary referral centre for paediatric and adult cardiac services in South Punjab. The data on all children below 15 years of age undergoing detailed transthoracic two-dimensional echo and Doppler studies was reviewed. Cases with isolated ventricular septal defects were studied for age of presentation, gender, type, and associated complications. The data was analyzed with SPSS 11 version. RESULTS: A total of 5018 patients with congenital heart diseases underwent echocardiography during this period. A total of 1276 patients had isolated VSD (25%). Mean age was 3.1 +/- 3.64 years (range: 1 day to 15 years). Females were 440 (34.5%) and males were 836 (66.5%). Of 1276 patients, 1014 (79.3%) were Perimembranous type, 124 (9.8%) were muscular type, 85 (6.7%) were doubly committed subarterial type and 53 (4.2%) inlet VSD. Small, moderate and large VSDs were 428 (33.6%), 443 (34.7%) and 405 (31.7%) respectively. Severe pulmonary hypertension was noted in 286 (22.4%) cases. Aortic valve prolapse was present in 85 (6.7%) cases and varying degrees of aortic valve regurgitation was seen in 67 (5.2 %) patients. Right ventricular outflow tract obstruction was found in 21 (1.6%) cases. Left ventricular outflow tract obstruction was noted in 09 (0.7%) cases. Echo evidence of infective endocarditis was present in 06 (0.5%) patients. CONCLUSION: Perimembranous ventricular septal defect was found to be the commonest type of ventricular septal defect. Large ventricular septal defects usually lead to severe pulmonary hypertension. Severe pulmonary hypertension was the commonest complication followed by Aortic Valve Prolapse and Aortic Regurgitation. Rest of the complications were rare.

An atypical cause of aortic valve prolapse.

We encountered a surgical case of aortic valve prolapse caused by a fissure formed in the commissural region and coronary cusp, and we achieved a favorable outcome. On pathologic examination, the fissured valve was found to be myxoid degeneration with ruptured elastic fibers and clustering of foamy macrophages, and it was diagnosed as an atherosclerotic change not associated with rheumatic change. The fissure appeared to develop because of hypertensive stress in a region rendered vulnerable by atherosclerosis. Although this mechanism is atypical, it should be recognized that poorly controlled hypertension can cause aortic valve prolapse and induce acute heart failure.

Natural aortic valve complications of ventricular septal defect: a prospective cohort study.

OBJECTIVE: To study the incidence and onset of aortic valve prolapse (AVP) and aortic regurgitation (AR) in the ventricular septal defect (VSD). STUDY DESIGN: A prospective cohort study POPULATION: The less than one-year-old children with diagnosis of isolated VSD were studied from October 2000 to September 2006 at Queen Sirikit National Institute of Child Health. Clinical follow-up and echocardiographic studies were scheduled every 2-3 months in the first year of age and then every 6 months to evaluate the size, location, flow across VSD, aortic valve morphology and aortic regurgitation. RESULTS: Three hundred and twenty-one cases of VSD were followed up. One was excluded due to associated hypoplastic RV An overall of 2,644 echocardiograms were performed. The percentage of perimembranous, subpulmonic, muscular inlet and multiple types were 70.3%, 19.4%, 5.6%, 3.1% and 1.6%, respectively. Size of the VSD was diagnosed to be small, moderate, and large VSD in 62.5%, 15.9% and 21.6% respectively. At the end of the study, the incidence of AVP in subpulmonic VSD was 87.1% compared to 16.4% in perimembranous VSD, with a relative risk of 5.30 and the incidence of AR in subpulmonic VSD was 37.1% compared to 5.3% in perimembranous VSD, with a relative risk of 6.95. From the survival analysis, the patient with subpulmonic VSD developed AVP at 46%, 77%, 90% and 94% compare to 8%, 13%, 20% and 23% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). The patient with subpulmonic VSD developed AR at 8%, 17%, 35% and 38% compare to 2%, 4%, 5% and 7% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). At the end of the study, ninety-six cases (30%) underwent cardiac operation with the indication of heart failure or the occurrence of AR. Sixty one cases (19.1%), including two cases of subpulmonic type had spontaneous closure of VSD. Seven cases (2.2%) had lost to follow up and five cases (1.6%) died during the follow up period. CONCLUSION: The incidence of AVP and AR are high in subpulmonic VSD being much higher than perimembranous VSD with a relative risk of 5.30 and 6.95 respectively. These complications are significantly from infancy period and are an indication for early cardiac surgery.

Left valsalva sinus aneurysm rupture into left atrium and aortic valve prolapse confirmed with transesophageal echocardiography.

We report a very rare case of left Valsalva sinus aneurysm rupture into left atrium in a 55-year-old man with severe aortic regurgitation caused by aortic valve prolapse. The Valsalva sinus aneurysm rupture was clearly visualized with transesophageal echocardiography performed to further delineate the aortic valve pathology. The 3-dimensional reconstruction of multislice computed tomography images nicely visualized the left Valsalva sinus aneurysm.

Frequency of development of aortic cuspal prolapse and aortic regurgitation in patients with subaortic ventricular septal defect diagnosed at <1 year of age.

The natural history of aortic cuspal prolapse and aortic regurgitation (AR), studied most commonly in subpulmonic ventricular septal defect (VSD), has not been well defined in isolated, unrepaired VSD diagnosed during infancy. This study aimed to define the incidence and progression of aortic cuspal prolapse and AR in patients with subaortic VSDs diagnosed at <1 year of age who had no aortic cuspal prolapse or AR at presentation and did not require surgery within the first year of life. Patients had yearly follow-up, and data regarding clinical course, physical examination, and echocardiography were obtained. Comparisons were made between patients who developed aortic cuspal prolapse and AR and those who did not. One hundred patients, with a mean age at VSD diagnosis of 0.1 +/- 0.5 years, followed for a mean of 7.1 +/- 10.1 years, were studied. Aortic cuspal prolapse developed in 14 patients (14%) at a mean age of 7.1 +/- 6 years (range 0.4 to 18.4). AR murmurs were heard in 6 patients (6%) at a mean age of 5.1 +/- 3.1 years, all of whom had aortic cuspal prolapse and underwent VSD closure and aortic valvuloplasty. In conclusion, aortic cuspal prolapse and clinical AR are not uncommon in patients with subaortic VSDs. Long-term follow-up of patients with subaortic VSDs should include the serial evaluation of aortic valve anatomy and function.

Traumatic aortic regurgitation caused by a detached commissure.

Case reports of traumatic aortic regurgitation caused by detached commissures are rare. We report a case of a 56-year-old man involved in a traffic accident. During his hospitalization for subdural hematoma and pulmonary contusion, he began to suffer from heart failure. He was operated on under diagnosis of severe aortic regurgitaion. The commissure between the left and the noncoronary cusps was largely detached from the aortic wall, which was easily estimated to be the cause of the prediagnosed left cusp prolapse. His aortic valve was replaced, and his postoperative course was uneventful.

Preservation of the bicuspid aortic valve.

BACKGROUND: Bicuspid anatomy of the aortic valve is a common reason for aortic regurgitation and is associated with aortic dilatation in more than 50% of patients. We have observed different patterns of aortic dilatation and used different approaches preserving the valve. METHODS: Between October 1995 and February 2006, a regurgitant bicuspid valve was repaired in 173 patients. The aorta was normal in 57 patients who underwent isolated repair. Aortic dilatation mainly above commissural level (n = 38) was treated by separate valve repair plus supracommissural aortic replacement. In 78 patients, aortic dilatation involved the root and was treated by root remodeling. RESULTS: Hospital mortality and perioperative morbidity were low in all three groups. Myocardial ischemia was significantly shorter in repair plus aortic replacement than remodeling (p < 0.001). Freedom from aortic regurgitation II or greater at 5 years varied between 91% and 96%. Freedom from reoperation at 5 years was 97% after remodeling, but only 53% after repair plus aortic replacement (p = 0.33). Symmetric prolapse was the most frequent cause for reoperation. CONCLUSIONS: The long-term stability of bicuspid aortic valve repair is excellent in the absence of aortic pathology. In the presence of aortic dilatation, root remodeling leads to equally stable valve durability. In patients with less pronounced root dilatation, separate valve repair plus aortic replacement may be a less complex alternative. Symmetric prolapse should be avoided if the ascending aorta is replaced.

Aortic insufficiency due to a partial left-coronary aortic valve prolapse and the detection of two small interatrial jets after blunt thoracic trauma.

Aortic valve regurgitation due to blunt thoracic trauma is a rare complication. Autopsy studies have been shown that the aortic valve is the most often lacerated one among the heart valves. Actually, we describe a case of a 47 year old man with the signs of heart failure after a blunt thoracic trauma 2 months before caused by aortic insufficiency due to a partial left-coronary aortic valve prolapse. Furthermore, transthoracic and transesophageal echocardiography revealed two small jets between the left and the right atrium.