SEARCH · Search PubMed
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Bicuspidization technique for rheumatic tricuspid stenosis based on the concept that the tricuspid valve has a straight (septal) and a curved (mural) leaflet is described. The fused commissures on either side of the septal leaflet are divided, resulting in separation of the two leaflets. The related anatomic and pathologic features are discussed.
A catheter-based intravascular ultrasound transducer was used to study aortic valve morphology in adults with calcific aortic stenosis. Examination of 14 postmortem specimens disclosed that intravascular ultrasound consistently identified the number of cusps or the presence of a calcified median raphe in the conjoined cusp, or both, and thereby distinguished a calcified bicuspid from a calcified tricuspid aortic valve. These postmortem findings were then employed to identify valvular morphology in 15 patients undergoing diagnostic cardiac catheterization or balloon aortic valvuloplasty, or both. Reproduction of criteria established in vitro allowed discrimination of congenital valvular morphology in all 15 patients, including 7 in whom assessment by intravascular ultrasound was confirmed by subsequent pathologic examination. Identification of aortic valvular morphology by intravascular ultrasound has potential therapeutic implications for patients considered to be candidates for operative or nonoperative aortic valvuloplasty.
OBJECTIVES: This study sought to determine the rate of familial occurrence of congenital bicuspid aortic valve (BAV) by using echocardiography to screen family members. BACKGROUND: Congenital BAV is a common anomaly that carries with it a significant risk of potential long-term cardiac complications. Despite several reports of the familial occurrence of BAV, the condition is not generally considered to be inherited. METHODS: Thirty consecutive patients with echocardiographically documented congenital BAV were interviewed to construct three-generation family pedigrees. All first-degree relatives were contacted to undergo echocardiography to specifically determine aortic valve morphology. RESULTS: Of the 210 first-degree relatives, 190 (90.5%) agreed to undergo echocardiography. Four members had technically difficult studies. Of the remaining 186 subjects, 17 (9.1%) were identified as having BAV; 11 (36.7%) of the 30 families had at least one additional member with the condition. The male/female ratio of affected members in the 11 families was 1. In one family, two instances of male-to-male transmission were observed. The distribution of BAV in the majority of multiplex families is compatible with autosomal dominant inheritance with reduced penetrance. CONCLUSIONS: We demonstrated a high incidence of familial clustering in congenital BAV. We believe that the high rate of occurrence of the condition in immediate relatives justifies echocardiographic screening of first-degree relatives to anticipate and prevent future complications associated with this common cardiac malformation.
Congestive heart failure developed in a 53-year-old man with a history of bicuspid aortic valve, angina, and pericarditis. Two-dimensional echocardiography showed a sinus of Valsalva aneurysm related to the anterior coronary sinus and color flow mapping identified a perforation of the anterior aortic cusp. These findings were confirmed during surgery.
Absent pulmonary valve syndrome (APVS) is an uncommon variant of tetralogy of Fallot (TOF), which manifests morphologically as vestigial pulmonary valve cusps at the right ventricle-pulmonary trunk junction. The aneurysmally dilated pulmonary arteries may compress the tracheobronchial tree and cause severe respiratory distress in the neonatal or infant stage. Early surgical correction in these patients is necessary despite the high operative mortality rate. A 1-day-old male neonate suffered from progressive shortness of breath after birth. Echocardiography confirmed the diagnosis of TOF with APVS. The marked dilatation of pulmonary arteries resulted in airway compression in addition to heart failure. Total surgical correction was performed at 40 days of age, using a homemade bicuspid equine pericardial tube for right ventricular outflow reconstruction. The short-term follow-up echocardiogram demonstrated good motility of the pericardial leaflet. However, patients receiving this type of valved conduit require meticulous long-term follow-up.
Cardiac anomalies are usually diagnosed early in life, which is particularly true for their various combinations. The diagnosis in adulthood is rare. Here we report the case of a young man with an aortic coarctation corrected at the age of 16, however the associated stenotic bicuspid aortic valve and cor triatriatum sinistrum were corrected after Streptococcus viridans endocarditis 7 years later.
INTRODUCTION: Chlamydia pneumoniae has been identified in arterial atherosclerosis. Aortic valves affected by senile calcific aortic stenosis (SCAS) or calcification of a congenital bicuspid valve (CCBAV) may have interior environments similar to atherosclerosis. This study aimed to detect C. pneumoniae within either SCAS or CCBAV. METHODS: 60 valves showing either SCAS (n=36) or CCABV (n=22) and control valves (n=2) were studied for the presence of C. pneumoniae by the following three techniques: (1) indirect immunofluorescence (IF) was performed on 36 SCAS valves, 22 CCBAV valves and 2 control aortic valves using a HEp-2 cell line infected with C. pneumoniae as a positive control. Negative controls comprised duplicate slides of the same valves with omission of the primary antibody step. A section of human stomach was also used as a negative control. A semiquantitative scoring method was used to grade positive IF staining. (2) Polymerase chain reaction (PCR) was performed on 30 SCAS valves, 20 CCBAV valves and 1 control valve using C. pneumoniae as a positive control and negative controls comprised a Ureaplasma sp. and human DNA from peripheral blood mononuclear cells. (3) Electron microscopy (EM) was performed upon 13 SCAS, 8 CCBAV and 2 control valves. RESULTS: All three methods failed to detect the presence of C. pneumoniae in any of the 60 aortic valves examined. False positive IF staining was encountered in 81% of test valves and in 76% of negative control valve sections (positive in calcified material due to nonspecific binding of FITC-conjugated secondary antibody). No staining was observed in the negative control stomach sections. CONCLUSIONS: This study failed to detect C. pneumoniae within aortic valves showing SCAS or CCBAV. Studies using IF alone to detect C. pneumoniae in calcified tissues should be interpreted with caution, since nonspecific binding of FITC-conjugated secondary antibody by calcium in the cusps may be misinterpreted as evidence of Chlamydia. The use of appropriate controls and ancillary methods for the identification of C. pneumoniae are important in this regard.
A 15-year-old boy presented with exertional palpitations and chest pain. Investigation revealed anomalous origin of his left anterior descending coronary artery from his pulmonary trunk causing myocardial ischaemia. He previously had aortic coarctation repair with known aortic root dilation and a bicuspid aortic valve. His left anterior descending artery was implanted into the aortic root using a Gortex interposition conduit. This represents an interesting combination of cardiac abnormalities for which repair required consideration of the requirement of further surgery in the future.
Noncompaction of the ventricular myocardium is a rare, unclassified cardiomyopathy due to an arrest of myocardial morphogenesis. The characteristic echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intertrabecular spaces communicating with the left ventricular (LV) cavity. The disease typically involves the LV myocardium, but right ventricular (RV) involvement is not uncommon. The clinical manifestations include heart failure (HF) signs, ventricular arrhythmias and cardioembolic events. Noncompacted myocardium may occur as an isolated cardiac lesion, as well as it can be in association with congenital anomalies. We describe two illustrative cases of noncompaction of the ventricular myocardium, a 19-year-old male with bicuspid aortic valve and progressive worsening of HF, and a 61-year-old male with marked RV involvement in addition to LV apical involvement, both with the typical clinical and echocardiographic features of the disease.
OBJECTIVE: Former studies have pointed out that hemodynamic stress imposed by associated valvular disease is the primary factor in the development of ascending aorta dilatation. At present, intrinsic wall pathology is blamed for dilatation and aneurysm formation in bicuspid aortic valve (BAV). MATERIALS AND METHODS: Aortic valve replacement (AVR) was performed on 78 adult patients with BAV. Patients were divided into two groups. Group I (n = 27) underwent only AVR. Group II (n = 51) underwent AVR and additional ascending aorta procedures such as Shawl-Lapel aortoplasty (n = 12) and tailoring aortoplasty (n = 9). Dacron wrapping was performed after both techniques were done. Ascending aorta replacement was done on 11 patients by using composite graft. Supracoronary graft replacement was performed in 3 patients after AVR. RESULTS: Ascending aorta diameter increment was 1.25 mm/year in normotensive and 2.80 mm/ year in hypertensive patients. Ascending aorta aneurysm (diameter > 55 mm) developed in eight patients in the postoperative period in group I. Ascending aorta dilatation did not develop in group II patients. Mean survival time +/- standard error (SE) was 128 +/- 11 and 99 +/- 4 months and survival possibility was 77.78% and 92.16%. Freedom from reoperation was 65.4% and 95.9% in 8 years in group I and group II, respectively. CONCLUSION: Aortic wrapping with or without aortoplasty has a beneficial effect not only in dilated ascending aorta but also in all nondilated BAV patients with normal-sized aortic diameter. Ascending aorta wrapping in BAV patients preserves the endothelial lining and prevents further dilatation, aneurysm formation, and dissection.
We report a case with echocardiographic demonstration of native congenital bicuspid aortic valve endocarditis with multiple subaortic complications. Transesophageal echocardiography in this case revealed large vegetations with multiloculated aortic paravalvular abscess around the cusps; a high-acquired restrictive membranous ventricular septal defect with vegetations extending to the tricuspid leaflets and paravalvular aortic regurgitation caused by aortic leaflet perforation.
We report the case of a 36-year-old man with calcified bicuspid aorta. Aortic disease was diagnosed after retinal embolism, which caused loss of vision affecting the left eye. Doppler examination did not identify carotid stenosis. Transthoracic echocardiography showed thickness and calcification of two leaflets, moderate aortic regurgitation, high normal left ventricular diastolic dimensions, and high normal interventricular septum thickness. Parasternal long-axis view in the systolic frame showed dilated left ventricular outflow tract, dilated ascending aorta, and calcification and thickening of aortic leaflets, causing an echo-lucent area, in the diastolic frame. It also showed the eccentric position of the closed valve leaflets, short axis in the diastolic frame, and the absence of the typical "Y" letter sign, tracked by the three leaflets (septal, right coronary, and left coronary) in the normal tricuspid aortic valve. On long-axis parasternal acquisition, we note high normal interventricular septum thickness and high normal diastolic diameter. Color Doppler analysis from the apical five-chamber view showed aortic regurgitation; regurgitant jet area was about 44% of the outflow tract, so aortic regurgitation was classified as moderate. Pulsed Doppler showed a high normal value of peak aortic velocity. Transesophageal echocardiography was performed, but no further findings were identified. We think that the likely retinal embolism sources were the aortic calcified leaflets, even if the aortic valve was not stenotic and with no signs of endocarditis. However, because we cannot exclude other potential causes, we put the patient on anticoagulant therapy. Furthermore, aortic valve replacement was not advised.
In three adult patients with diseased congenital bicuspid aortic valves and aneurysms of the ascending aorta, we performed aortic valve replacements, and combined a proximal and middle vertical reduction aortoplasty with a distal external synthetic wrapping. During a mean follow-up of 23.3 months there is no evidence of compression of the innominate artery by the distal external synthetic wrap, and no redilatation of the ascending aorta.
BACKGROUND: A congenital bicuspid aortic valve (BAV) in adults is known to be associated with aortopathy, specifically with aneurysms of the ascending aorta (AA). MATERIALS AND METHODS: Ten (16.7%) patients with congenital BAV had an aneurysm of the AA. Aortic valve replacement was performed in 58 patients, repair in two. Aneurysms were repaired by a vertical reduction (tailoring) aortoplasty (VRA) with or without external synthetic grafting (wrapping, wrap) in nine patients, replaced in one. RESULTS: There were no operative deaths. One patient died 87 months after the operation due to a noncardiac cause. Fifty-four patients are alive and well, without the evidence of recurrence of an aneurysm. CONCLUSIONS: A VRA with or without external synthetic wrapping appears to be a satisfactory alternative to replacement procedures for the AA aneurysm in older patients with BAV, and those with comorbid conditions.
An adult patient with a congenital bicuspid aortic valve, an aneurysm, and a calcific atherosclerotic plaque of the ascending aorta, underwent an aortic valve replacement, a vertical reduction aortoplasty, an endarterectomy, and a distal external synthetic wrapping.
A 59-year-old white male presents to our hospital with shortness of breath, bilateral lower extremities swelling, an ejection systolic murmur, and S4 gallop. Echocardiography revealed a congenital bicuspid aortic valve (BAV). Subsequent coronary angiogram revealed a single coronary artery arising as a main trunk from the left coronary sinus. We report the second case of a single coronary artery and congenital BAV.
A 6 month Welsh pony filly with an interventricular septal defect in conjunction with a pulmonary stenosis due to a bicuspid pulmonary valve is described. The animal had poor exercise tolerance, a loud pansystolic murmur and a precordial thrill. Blood pressure and oxygen tension values obtained during cardiac catheterization suggested the diagnosis which was confirmed at autopsy.
OBJECTIVES: To describe the incidence and rate of dilatation of the ascending aorta in children with bicuspid aortic valve (BAV) and to determine factors that predict rapid aortic dilatation. DESIGN: Retrospective cohort study. SETTING: Regional tertiary care children's hospital. PATIENTS: All children aged 0-18 years seen at the authors' institution between 1990 and 2003 with an "isolated" BAV. All patients had had more than one technically adequate echocardiogram, at least six months apart, with concomitant height and weight data. INTERVENTIONS: Offline echocardiographic measurements of multiple levels of the aortic root were completed for each participant at each serial echocardiogram. These measurements were then compared with expected measurements derived from a normal local control population. MAIN OUTCOME MEASURES: Rate of change of the ascending aorta size over time, where aortic size is expressed as the number of standard deviations above or below the mean size expected for a given body surface area (z score). RESULTS: 279 echocardiograms spanning a period of from 9 months to 13.3 years were analysed for 88 patients with BAV. The ascending aorta in the BAV group was larger than expected for body surface area at diagnosis and continued to increase in relative size at each of the four subsequent follow-up echocardiograms. Ascending aortic z score increased at an average rate of 0.4/year. A faster rate of increase in z score was predicted by both larger initial aortic valve gradient and non-use of beta blockers. CONCLUSIONS: Children with BAV are at risk of having a dilated ascending aorta. This risk increases with longer follow up.