Progression of aortic valve dysfunction in 51 adult patients with congenital bicuspid aortic valve: assessment and follow up by Doppler echocardiography.

OBJECTIVE--To assess the pattern and progression of aortic valve dysfunction by serial Doppler echocardiographic examinations in ambulatory adult patients with congenital bicuspid aortic valve. DESIGN AND SETTING--Retrospective analysis of patients referred for Doppler echocardiography over a four year period. SUBJECTS--Fifty one adult patients with echocardiographic diagnosis of congenital bicuspid aortic valve had serial Doppler echocardiographic studies at least six months apart. There were 40 men and 11 women with a mean age of 36 years. MAIN OUTCOME MEASURE--Doppler echocardiographic values of aortic valve dysfunction. Cardiac events including endocarditis and aortic valve replacement were also evaluated. RESULT--Coarctation was present in five patients. 31 (61%) patients had a functionally normal bicuspid aortic valve defined as a mean gradient < 25 mm Hg and mild regurgitation. Significant aortic regurgitation was present in 15 patients (moderate in 12 and severe in three). Three patients had isolated aortic stenosis and two patients had combined aortic valve dysfunction. At a median follow up of 21 months (range six to 46 months), six patients had aortic valve surgery (one for aortic stenosis, three for aortic regurgitation, and two for endocarditis). Only 22 patients (43%) continued to have a functionally normal aortic valve. CONCLUSION--In this cohort of fairly young patients, aortic regurgitation is more common than aortic stenosis. Progression of aortic valve dysfunction occurs in patients with pre-existing valve dysfunction and even in those with normal aortic valve function at the initial echocardiographic examination.     

A case of subannular aortic aneurysm detected by transoesophageal echocardiography.

Subvalvular aortic aneurysm is a cardiac abnormality extremely uncommon in white adult patients. Accurate diagnosis can be made by transoesophageal echocardiography which is the modality of choice for non-invasive recognition of aortic and subaortic abnormalities. In this report we describe, in a 47-year-old white man with subvalvular aortic aneurysm associated to aortic bicuspid valve, the echocardiographic diagnosis and cardiac intervention of aortic valve replacement with surgical obliteration of the orifice. A good surgical result was confirmed by an echocardiographic transthoracic examination performed after 6 months.     

Acute aortic regurgitation due to spontaneous rupture of a bicuspid aortic valve: detection by echocardiography

A 42-year-old man was admitted with acute severe aortic regurgitation. There were no signs of a systemic infection. M-mode and two-dimensional echocardiography revealed bicuspid aortic valve and echocardiographic features consistent with aortic leaflet rupture. The diagnosis was confirmed at surgery. This report illustrates that spontaneous rupture of a bicuspid aortic valve should be considered in acute aortic regurgitation without infective endocarditis.     

Abnormal aortic valve echoes in mitral prolapse. Echocardiographic features of floppy aortic valve.

The following distinctive combination of M-mode and two dimensional echocardiographic abnormalities of the aortic valve was observed in a group of 12 patients, of whom 11 had mitral valve prolapse. On two dimensional scans, the aortic cusps were freely mobile but appeared thickened or folded. On M-mode records, cusp excursion was normal: there was well defined systolic oscillation, and cusp echoes were multiple and centrally positioned within the aortic root during diastole. The aortic valve was inspected at operation in two patients: a typically myxomatous valve was replaced in one and findings were in keeping with this diagnosis in the other. It is suggested that the echocardiographic features described are characteristic of the floppy aortic valve. Despite the echocardiographic abnormalities, only three patients had clinical evidence of an aortic valve lesion. It is, therefore, further suggested that the investigation of patients with mitral prolapse should include echocardiographic assessment of the aortic valve, even when associated myxomatous degeneration of that valve is not suspected clinically.     

Comparison of M mode echocardiography and pathologic findings in the hypoplastic left heart syndrome

The M mode echocardiograms and pathologic specimens from 18 infants with the hypoplastic left heart syndrome were compared to determine the accuracy with which the echocardiogram reflects the state of the mitral valve, left ventricle and aortic valve, and its limitations in establishing this diagnosis. Mitral valve echoes were recorded in 7 of the 11 cases with an anatomic mitral valve orifice diameter greater than 3 mm. Ventricular septal echoes were found only in the seven cases in which the mitral valve was recorded. Differences between echocardiographic and anatomic left ventricular internal dimensions were not statistically significant in this small group. When aortic valve echoes were recorded, this valve was always patent in the anatomic specimen. The aortic valve was atretic in 9 of the 11 cases in which no aortic valve echoes were present. The echocardiographic and anatomic aortic root dimensions had a statistically significant correlation (p < 0.05), but there was considerable scatter in the data.None of the 18 infants met all of the previously proposed criteria for the echocardiographic diagnosis of hypoplastic left heart syndrome. An echocardiographic left ventricular dimension of 10 mm or greater was present in five cases (28 percent), and an aortic root dimension of 10 mm or greater in six (33 percent). The most reliable echocardiographic finding was excursion of 5 mm or less of the anterior leaflet of the mitral valve or inability to detect the mitral valve. Noninvasive findings are not always sufficient to establish the diagnosis of hypoplastic left heart syndrome, and further studies may be necessary in some patients.     

Echocardiography of the "floppy" aortic valve. Report of a case.

The echocardiographic features of a patient with severe aortic regurgitation due to a "floppy" aortic valve are presented. The salient abnormality observed was marked fluttering of the aortic valve cusps in diastole. Fluttering of the anterior leaflet of the mitral valve and left ventricular enlargement were noted. The diagnosis of a "floppy" aortic valve was substantiated at surgery.     

Left ventricular outflow tract obstruction from mitral prosthesis

A case of left ventricular outflow tract obstruction six years following mitral valve replacement is presented. Clinical features, echocardiographic and angiographic studies preoperatively all suggested aortic valve disease. The diagnosis of left ventricular outflow tract obstruction by the strut of the mitral prosthesis was made intraoperatively. Enlargement of the aortic root and aortic valve replacement resulted in good symptomatic and echocardiographic improvement.     

A Case of Late Presenting Unicuspid Aortic Valve in a 62‐Year‐Old Female

We describe the case of a 62‐year‐old female presenting with new onset progressive dyspnea on exertion and a new diagnosis of aortic stenosis. Transesophageal echocardiography showed fusion of the aortic valve leaflets suspicious for bicuspid aortic valve with dilated ascending aorta. Surgical replacement of the valve revealed a unicuspid aortic valve with one true commissure at the level of the left and noncoronary cusps. The case is supported by clinical, echocardiographic and angiographic imaging and discussion of current imaging modalities used in diagnosis of aortic valve pathology.     

Two dimensional echocardiographic assessment of aortic valve morphology: feasibility of bicuspid valve detection. Prospective study of 100 adult patients.

Two dimensional echocardiographic criteria for bicuspid aortic valve recognition have greater specificity than previously proposed M-mode echocardiographic criteria. The potential clinical use of the two dimensional technique is, however, limited by the technical inability to image adequately the aortic valve leaflets in many patients. One hundred consecutive adult patients undergoing two dimensional echocardiography were prospectively studied. Valve cusp number could not be determined because of dense calcification in eight patients. A bicuspid aortic valve was diagnosed in a single subject. A parasternal short axis view disclosed three commissures (diastolic "Y" configuration) in only 26 patients. Technically adequate parasternal short axis imaging was more likely in younger patients and in non-smokers. In patients not successfully imaged from the parasternal approach, an anteriorly tilted apical four chamber view showed a diastolic "Y" configuration in an additional eight cases. Considering the high prevalence in our population of incomplete two dimensional echocardiographic aortic valve leaflet imaging, angiographic and/or pathological studies must be performed to establish the correlation between these incomplete echocardiographic patterns and aortic valve anatomy if two dimensional echocardiography is to have widespread application in the diagnosis of the congenital bicuspid aortic valve.     

Role of transoesophageal echocardiography in the diagnosis and management of aortic root abscess.

OBJECTIVE--To assess and compare the roles of transthoracic and transoesophageal echocardiography in the diagnosis and management of an aortic root abscess. DESIGN--To select patients with echocardiographic diagnosis of aortic valve endocarditis with and without an aortic root abscess and correlate this with a retrospective review of surgical and necropsy data. SETTING--Tertiary referral centre at a university teaching hospital. PATIENTS AND METHODS--34 patients with confirmed aortic valve endocarditis were treated over a four and a half year period. All patients underwent both transthoracic and transoesophageal echocardiography with 17 patients having biplane or multiplane imaging. RESULT--11 patients (32%) had an aortic root abscess. Transthoracic echocardiography identified four cases of aortic root abscess whereas transoesophageal echocardiography correctly detected all 11 cases and also detected complications including mitral aortic intervalvar fibrosa fistula in two patients and right atrial involvement in another two patients. Only biplane imaging was able to show an anterior aortic root abscess in one patient and the circumferential involvement of the aortic annulus in another two patients. All patients with an aortic root abscess were treated surgically after transoesophageal echocardiographic diagnosis. After operation, prosthetic aortic regurgitation was present in seven patients and a repeat operation was performed in three patients. Only transoesophageal echocardiography detected a postoperative aorto-right atrial fistula in two patients and recurrence of the root abscess in another. There were five deaths in hospital (45%). CONCLUSIONS--Compared with transthoracic echocardiography, transoesophageal echocardiography was more sensitive and more specific for the early diagnosis of aortic root abscess and its complications and facilitated both the preoperative and postoperative management of these patients. Biplane and multiplane imaging provide additional diagnostic information. All patients with suspected aortic valve endocarditis should have an early transoesophageal echocardiographic study.     

Echocardiographic manifestations of valvular vegetations

Eight patients with autopsy or surgically proved valvular vegetations were examined using echocardiography. Five of these patients had lesions on the aortic valve and three had lesions on the mitral valve. The echocardiographic finding in these patients was a non-uniform thickening of valve leaflets which exhibited unrestricted motion. Often the abnormal echoes which produced the thickened valve had a shaggy appearance. In all eight patients the location of the echocardiographic abnormality correlated with the anatomic findings at surgery or autopsy. In one patient the diagnosis of bacterial endocarditis was first suspected following the echocardiographic examination and only subsequently was a heart murmur heard. These findings indicate that echocardiography may play a useful role in elucidating the pathological anatomy of the bacterial endocarditis with vegetation; however, the length of time from the onset of clinical illness to echocardiographic diagnosis remains unknown.     

Aortic valve prolapse with aortic regurgitation assessed by Doppler color-flow echocardiography.

The incidence of and the Doppler color-flow echocardiographic characteristics of aortic valve prolapse with nonrheumatic aortic regurgitation were examined. Aortic valve prolapse was observed in 21 of 243 patients (15 men and 6 women) with aortic regurgitation as detected by Doppler color-flow echocardiography (rheumatic, 112; nonrheumatic, 131) in 1247 consecutive patients. Patients with aortic valve prolapse included three patients with essential hypertension and one with annuloaortic ectasia. The remaining 17 patients (7% of those with aortic regurgitation) had no other associated cardiovascular disease (idiopathic aortic valve prolapse). Prolapse of the mitral or the tricuspid valve or both was associated with aortic valve prolapse in seven patients. Aortic regurgitation jet was markedly deviated from the axis of left ventricular outflow tract toward the anterior mitral leaflet or the interventricular septum in 17 of 21 (81%) patients with aortic valve prolapse, whereas 28 of 110 (25%) patients with nonrheumatic aortic regurgitation without prolapse and 17 of 112 (15%) patients with rheumatic aortic regurgitation without prolapse showed the deviation of regurgitant jet (p < 0.001). In conclusion, idiopathic aortic valve prolapse is one of the significant causes of aortic regurgitation, and a marked deviation of regurgitant jet is a characteristic Doppler color-flow echocardiographic finding of aortic regurgitation that results from aortic valve prolapse.     

Evaluation of ejection murmurs by pulsed Doppler echocardiography.

A common problem is a soft basal ejection murmur in an asymptomatic child with a normal electrocardiogram and chest x-ray films. If the diagnosis is aortic stenosis, there is a need for prophylaxis for subacute bacterial endocarditis and concern about development of calcific aortic stenosis. In 40 consecutive children referred for this differential diagnosis, aortic stenosis was diagnosed in 30, based on an ejection murmur at the second right interspace (not necessarily louder than at the second left), which transmitted well to the neck, accompanied by a normal second heart sound. Pulsed Doppler echocardiography confirmed turbulence at the aortic valve in 26. In the 10 children diagnosed clinically as having an innocent murmur, three had evidence on pulsed Doppler echocardiography for an abnormal aortic valve. The conventional echocardiographic findings of an eccentric aortic orifice were present in only 21 of 29 patients with aortic turbulence. Though our current clinical criteria are reasonably specific (87%), they are not as sensitive (70%). Pulsed Doppler echocardiography provides powerful non-invasive assistance for this important differential diagnosis.     

Prosthetic Aortic Valve Thrombosis Detected by Doppler Echocardiography

Thrombosis of the prosthetic valve is a highly lethal medical emergency that requires immediate diagnosis and prompt therapy. Establishing the diagnosis may be difficult, despite numerous physical, echocardiographic, fluoroscopic, and angiographic signs, which have been described. We report a case of prosthetic aortic valve thrombosis diagnosed by Doppler echocardiography.     

Fungal endocarditis complicated by mycotic aneurysm of sinus of valsalva,interventricular septal abscess,and infectious pericarditis: Unique m-mode and two-dimensional echocardiographic findings

A 26-year-old male with fungal aortic endocarditis is presented in whom unique M-mode and two-dimensional echocardiographic findings permitted a diagnosis of mycotic aneurysm of right sinus of Valsalva and ventricular septal abscess preoperatively. The aneurysm was excised, and the affected aortic and tricuspid valves were replaced with valve prostheses.     

Infective endocarditis of aortic valve during pregnancy: a case report

Infective endocarditis during pregnancy is uncommon but very serious. A 31-year-old woman in the 36th week of second pregnancy was admitted to a hospital because of fever, weakness, chest pain, painful skin over her right leg and dyspnea. Transthoracic echocardiography showed aortic valve vegetation and severe aortic regurgitation. Transesophageal echocardiography revealed a 18 mmx6 mm mobile vegetation, attached to the right coronary cusp. Emergency cesarean section followed with a delivery of a healthy baby. Cardiopulmonary bypass with subsequent aortic replacement with bioprosthesis was initiated immediately after cesarean section. Early echocardiographic examination and 6 months after surgery revealed normal function of aortic valve bioprosthesis and normal LV function. Clinical recognition and early echocardiographic diagnosis followed urgent simultaneous cesarean section and aortic valve replacement was lifesaving for both mother and fetus.     

Aortic and Mitral Valve Stenosis with Regurgitation: Not Due to Rheumatic Heart Disease

The patient is a 13‐year‐old Mexican–American immigrant who had no previous medical care. Upon arrival to the United States she was diagnosed with severe mitral valve stenosis and regurgitation. In addition she had severe aortic stenosis with mild to moderate aortic valve regurgitation, which was thought to be rheumatic heart disease. On the basis of the clinical and echocardiographic findings she was taken to the operating room for both mitral and aortic valve replacement. Her operation was complicated by the discovery that her aorta was completely calcified, as were her coronary arteries, mitral valve and aortic valve. She underwent aortic and mitral valve replacement as well as replacement of her aortic arch. Her coronary arteries were patch augmented and reimplanted into the newly created ascending aorta. Based on the pathologic examination of the surgical tissue a diagnosis of Gaucher disease was made. (Echocardiography 2011;28:E24‐E27)     

Reliability of transthoracic echocardiography in the assessment of aortic valve morphology: pathological correlation in 178 patients

OBJECTIVE: To examine patient characteristics affecting the utility of transthoracic echocardiography in determining aortic valve morphology, particularly for the diagnosis of congenital bicuspid aortic valve (BAV). DESIGN: A retrospective comparison of preoperative echocardiographic determination of aortic valve morphology with pathological findings of the explanted valves. SETTING: A tertiary referral centre. PATIENTS: Consecutive patients who had aortic valve replacement between July 1994 and April 1996, and had preoperative echocardiograms. RESULTS: Of 313 patients, 181 (58%) had preoperative echocardiography. Three of the valves were excluded because they were too fragmented for pathological determination of valvular morphology. In the remaining 178 patients, aortic valvular morphology was determined by echocardiography in 104 (58%). Multivariate analysis showed that echocardiography was successful less often in women (odds ratio 0.44, P = 0.03) and in patients with densely calcified valves (odds ratio 0.69, P = 0.02), whereas age had no effect (odd ratio 0.99, P = 0.42). In those with adequate echocardiographic images, echocardiography had both a high sensitivity (0.92) and a high specificity (0.96) for the diagnosis of BAV. CONCLUSIONS: Echocardiography is a useful tool for the diagnosis of BAV, although suboptimal images may pose a problem in many patients, particularly women and patients with heavily calcified valves. When adequate images are obtained, transthoracic echocardiography can reliably identify aortic valvular morphology in most patients.     

Quadricuspid Pulmonary Valve Identified by Transthoracic Echocardiography

The quadricuspid pulmonary valve is a rare congenital anomaly. It tends to be clinically quiescent. Its diagnosis by two-dimensional echocardiography could be very difficult because of the anatomical features. We report on the echocardiographic findings of a 66-year-old female patient with mitral and aortic regurgitation of rheumatic origin and severe pulmonary hypertension. There was an aneurismatic dilation of the pulmonary artery trunk allowing visualization of the short-axis view of the pulmonary valve. It showed four cusps of similar size and an important deficit of central coaptation. Very few cases of the quadricuspid pulmonary valve are documented in live patients .     

Flail aortic valve leaflets: M-mode and two-dimensional echocardiographic manifestations

Four patients with documented flail aortic valve leaflets were studied using M-mode and two-dimensional echocardiography (2-D echo). Two had aortic valve endocarditis, one had endocarditis involving a congenital heart defect, and one had a myxomatous aortic valve. Mitral valve flutter and early mitral valve closure led to the diagnosis of severe aortic insufficiency in three patients. Diastolic aortic valve flutter, considered to be specific for a flail aortic leaflet, was present in three patients. In the fourth patient left ventricular outflow tract (LVOT) echoes were present, but did not distinguish between a flail aortic leaflet and an aortic vegetation. Two-D echo confirmed LVOT echoes in all patients. Discrimination between a flail leaflet and a vegetation(s) without leaflet disruption was accomplished by noting the hinge point of the LVOT diastolic echoes, which was the aortic wall in patients with a flail leaflet.The combination of these M-mode and 2-D echocardiographic findings permitted the diagnosis of a flail aortic leaflet to be made accurately and noninvasively. In two patients surgery was performed without prior cardiac catheterization.