Quadricuspid Valve Research Articles

Aortic Valve Disease in Pulmonary Atresia and Major Aortopulmonary Collaterals

Aortic valve disease in association with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (PA/VSD/MAPCAs) is an extremely rare combination of congenital heart defects. The presence of aortic stenosis or insufficiency or both imposes an additional physiologic burden that complicates the management of PA/VSD/MAPCAs. This report summarizes our experience with 7 patients who underwent surgical repair of this rare combination of defects. This was a retrospective review of patients who had both aortic valve disease and PA/VSD/MAPCAs. Four patients had predominantly aortic stenosis, 2 patients had aortic insufficiency, and 1 had mixed stenosis and insufficiency. Anatomically, this corresponded to a dysplastic trileaflet valve in 4, bicuspid valve in 2, and quadricuspid valve in 1. Four of the 7 patients underwent aortic valve surgery at the time of their first operation for PA/VSD/MAPCAs. The remaining 3 patients had aortic valve surgery at subsequent operations. All 7 patients are alive, with a median duration of follow-up of 3 years. One patient has required aortic valve replacement 2 years after repair of an insufficient bicuspid valve. This manuscript summarizes our experience with 7 patients who had the rare combination of aortic valve disease and PA/VSD/MAPCAs. Although aortic valve disease has been reported for other conotruncal defects, we believe this is the first report of its presence in association with PA/VSD/MAPCAs.

Surgical repair of truncal valve regurgitation†

Truncal valve regurgitation remains a short- and long-term risk factor for patients with truncus arteriosus. There are limited data available on techniques and outcomes of truncal valve repair (TVR). The aim of this study was to report our experience with TVR in patients of all ages. From 1997 to 2012, 36 patients (13 neonates, 30 children and 3 adults) underwent TVR for significant regurgitation. There were 3 early deaths (8%), all of which were in neonates. Twenty-two patients had a quadricuspid, 13 a tricuspid and 1 a bicuspid truncal valve before repair. Valve repair improved regurgitation in 31 of 36 repairs. The median regurgitation decreased from moderate-severe to mild (P < 0.001). During a mean follow-up of 38.3 ± 44.9 months (range 1 month-15 years), there was 1 late death, 16 patients required reoperation on the truncal valve and 1 required a second reoperation. Freedom from reoperation was 91.4 ± 4.8% at 1 year, 55.0 ± 10.4% at 5 and 22.9 ± 12.2% at 10 years. A quadricuspid valve after repair tended to worsen freedom from reoperation (P = 0.15), and tricuspidization tended to improve freedom from reoperation (P = 0.19). Neonatal repair (hazards ratio (HR) 4.1, P = 0.03) and leaflet thinning (HR 22.5, P = 0.002) were independent predictors of reoperation. Valve repair for truncal valve regurgitation is feasible, with good results. Surgical creation of a tricuspid truncal valve seems to provide the best outcomes in this challenging population.

Massive aortic regurgitation in the background of a quadricuspid aortic valve

A 52-year-old woman, with a previous history of hypertension, was admitted to our institution presenting exertional dyspnea. A comprehensive study was performed including a cardiology examination and echocardiography. Transesophageal echocardiography detected a quadricuspid aortic valve with a central severe aortic valve insufficiency. Moreover, a discrete dilation of the aortic root was seen. Left ventricular ejection fraction was in normal range. A computed tomography scan was performed especially to recognize any displacement of the coronary ostia and evaluate the aortic valve (Fig. A). No abnormality of coronary artery origin was confirmed. Surgical treatment was proposed, and the patient underwent open-heart surgery. Quadricuspid valve was confirmed (Fig. B). An aortoplasty to reduce the ascending aorta and a mechanical aortic valve replacement were performed. The postoperative course was favorable, and the patient had an uneventful recovery. Postoperative echocardiography assessment confirmed normal prosthetic aortic valve function and left ventricular ejection fraction. Quadricuspid aortic valve is a rare congenital abnormality, with an estimated incidence of 0.04%. In approximately 80-90% of cases, it is related to an aortic valve dysfunction. Quadricuspid aortic valve is usually associated with additional malformations, mainly related to the origin of coronary arteries. Due to the presence of an additional leaflet, absence or displacement of coronary ostia has been found in 10% of cases. It is of utmost importance to prevent coronary ostia obstruction with valve prosthesis implantation, and to plan myocardial protection during cardiopulmonary bypass. In the presence of prior surgery for congenital aortic valve abnormalities and regardless of patient age, we highlight the necessity to perform a careful evaluation of the coronary anatomy. 780

Leonardo da Vinci and the first portrayal of quadricuspid semilunar valves and the nodules of Arantius

Leonardo da Vinci and the first portrayal of quadricuspid semilunar valves and the nodules of Arantius

Dynamic Evaluation of a Quadricuspid Aortic Valve With Coronary Computed Tomography Angiography

A 35-year-old man presented with 9 years of chronic chest pain and was found to have moderate-to-severe aortic regurgitation on echocardiography. Aortic valve morphology on the initial echocardiogram was deemed normal. Computed tomography angiography was obtained for further evaluation of the aortic root dimension and anatomy. In addition to noninvasive evaluation of the coronary arteries, the higher spatial resolution and volumetric coverage of computed tomography angiography can better define the valvular and aortic anatomy. Reconstruction of retrospectively gated cine images through the aortic valve plane revealed a quadricuspid valve with 4 equal-sized cusps. Incomplete coaptation of the aortic valve cusps was seen during diastole, explaining the marked aortic regurgitation. In addition, a dilated ascending thoracic aorta (4.7 cm) was revealed. On the basis of these findings, the patient was treated surgically with a composite valve graft replacement of his aortic root.

Aortic valve reconstruction using self-developed aortic valve plasty system in aortic valve disease

Aortic valve disease is usually treated by prosthetic valve replacement. We have performed aortic valve plasty (AVP) using glutaraldehyde-treated autologous pericardium. AVP was performed for 88 patients from April 2007 through August 2009. Sixty-five patients had aortic stenosis, and 23 patients had aortic regurgitation (AR). Twenty-one patients showed bicuspid aortic valves, and one patient showed quadricuspid valve. There were 43 males and 45 females. Their mean age was 70.6±10.5 years old. First, diseased leaflets excised. Then, the distance between each commissure was measured. The new leaflet were trimmed with an original template from a glutaraldehyde-treated autologous pericardium sample. Finally, the annular margin of the pericardial leaflet was running sutured to each annulus. There was no operative mortality or embolic event. Postoperative echocardiography revealed a mean peak pressure gradient (PG) of 19.0±9.1 mmHg one week after surgery. Thirty-two patients had echocardiography one year after surgery. The peak PG became 12.9±5.8 mmHg. Ten patients showed no AR, 20 patients showed trivial AR, and two patients showed mild AR. Freedom from reoperation is 100% at three years follow-up.

Quadricuspid valve: External volume rendering view using dual-source computed tomography

Quadricuspid valve: External volume rendering view using dual-source computed tomography

Bicuspid aortic valve: differences in the phenotypic continuum affect the repair technique☆

Bicuspid aortic valve (BAV) may be considered in the context of a phenotypic continuum of the aortic valve configuration, ranging from severe forms of unicuspid valve to rare forms of quadricuspid valve. In this article, we report our results with BAV repair using surgical techniques tailored to the specific features of the BAV phenotypic continuum. Between September 2003 and May 2009, 31 patients with BAV (mean age 49.9+/-17.3 years; five female; 26 male) were prospectively enrolled for aortic valve repair. The surgical strategy was tailored depending on the anatomical and structural characteristic of the BAV continuum: type 1 real bicuspid (five patients), type 2 bicuspid with raphe (24 patients) and type 3 clefted bicuspid (two patients). An echocardiographic analysis was performed preoperatively, postoperatively, at discharge and every 6 months during follow-up. Kaplan-Meier analysis was used to assess the freedom from re-operation. One patient died in the intensive care unit due to aortic wall rupture. After BAV repair there was an increase of leaflet coaptation length (from 2.0+/-0.5 mm to 8.2+/-1.9 mm, p<0.01) and a decrease of the diameters of the virtual basal ring (from 24.6+/-3.6 mm to 21.5+/-0.71 mm, p<0.01), of the aortic root (from 43.12+/-13.23 mm to 31.0+/-3.2 mm, p<0.01) and of the sino-tubular junction (from 40.1+/-6.95 mm to 33.5+/-9.19 mm, p<0.05). The aortic regurgitation echocardiographic grade > or =II was found preoperatively in 30 patients and postoperatively in one patient (p<0.01). Six patients underwent an associated aortic valve-sparing procedure (three re-implantation and three remodelling). Kaplan-Meier analysis showed a 96.6% freedom from re-operation at 5 years, with a single new repair procedure and a 100% freedom from aortic valve replacement. BAV repair provides a feasible therapeutic option in selected patients with good medium-term results. The surgical techniques should be applied after a careful analysis of the BAV considered in the context of the phenotypic continuum.

Valva aórtica quadrivalvular por ecocardiografia transtorácica

A 55-year-old woman seeks medical attention with palpitations, atypical chest pain and dyspnea upon exertion, lasting for 12 months. ECG showed left ventricular hypertrophy. Transthoracic Doppler echocardiogram confirmed hypertrophy of the left chambers and showed a quadricuspid aortic valve, with equal-sized cusps, in addition to a moderate regurgitant central flow. Upon echocardiography, the quadricuspid valve is identified by its characteristic "X" form during diastole and rectangular aspect during systole. Heart failure symptoms are presumably caused by valve insufficiency due to abnormal leaflet coaptation. Considering the possibility of progressive regurgitation, follow up on the patient was performed with periodic echocardiographic control. Symptoms responded to treatment.

Unicuspid aortic valve mimicking quadricuspid valve

Unicuspid aortic valve is a rare congenital cardiac abnormality, leading to aortic stenosis or regurgitation. We report the case of a 55-year-old man with severe aortic regurgitation caused by a unicuspid valve mimicking quadricuspid valve.

Aortic Valvar Atresia, Interrupted Aortic Arch, and Quadricuspid Pulmonary Valve: A Rare Combination

Aortic atresia and interrupted aortic arch is a rare cardiac combination. Review of the literature revealed nine cases. We present two patients with this combination and the additional finding of quadricuspid pulmonary valves, one of which was severely stenotic. In the latter patient, an aortopulmonary window was present. The other had a unique blood supply to the brachiocephalic arteries and ascending aorta from systemic collateral arteries. To the best of our knowledge, the association of a quadricuspid pulmonary valve with this combination has not been previously reported.

Anatomic relationship of the coronary orifice and truncal valve in truncus arteriosus and their surgical implication

350 The Journal of Thoracic and Cardiovascular Surgery • February 2002 A trileaflet configuration is considered essential to maintaining the competence of a chorda-less semilunar valve.1 Bicuspid aortic and pulmonary valves are prone to stenosis, and quadricuspid valves are susceptible to regurgitation. Truncal insufficiency occurs more frequently in quadricuspid than tricuspid valves, but no cases have occurred in bicuspid valves.2 One method of restoring competence in truncal insufficiency is to excise one of the quadricuspid truncal valves (QTV) with its wall.2 To use that supernumerary cusp and its truncal wall, we reviewed the reported truncal valve anatomy in relation to the coronary orifice and conceived the following procedure for reconstruction.

Coronary arteries in truncus arteriosus

The origin and distribution of the coronary arteries was described in 39 autopsy specimens of truncus arteriosus (TA). The specimens were classified according to the number and the patterns of the truncal cusps. The position of the truncal cusps was defined in relation to intracardiac structures, namely, the atrioventricular orifices. Bicuspid truncal valves were observed in 8 cases (21%), tricuspid in 22 cases (56%) and quadricuspid in 9 cases (23%). All tricuspid valves had 2 anterior and 1 posterior cusp. Great variability in the origin of the coronary arteries was observed, with a tendency for the right coronary artery to arise from the anterior right quadrant and for the left coronary artery to arise from the anterior and left quadrant. Such a tendency was observed in 50% of the bicuspid, in 59% of the tricuspid and in 66% of the quadricuspid valves. The anatomic right ventricle was always observed to be vascularized by a right coronary artery, and the anatomic left ventircle by a left coronary artery, even in cases in which there was a single coronary trunk. The anterior surface of the right ventricle was crossed by a right coronary artery in 5 cases. A single coronary artery was observed in 7 cases (18%). Embryologic considerations are offered, especially regarding the relation between the observed variability in coronary artery patterns in TA and the absence of the truncal septation.

Rare or unusual causes of chronic, isolated, pure aortic regurgitation

Six patients undergoing aortic valve replacement had rare or unusual causes of isolated, pure aortic regurgitation. Two patients had congenitally bicuspid aortic valves with a false commissure (raphe) displaced to the aortic wall ("tethered bicuspid aortic valve"), two had floppy aortic valves, one had a congenital quadricuspid valve, and one had radiation-induced valve damage.

22 先天性要因による成人大動脈弁疾患に対する人工弁置換術の成績

22 先天性要因による成人大動脈弁疾患に対する人工弁置換術の成績