Supravalvular Ring Research Articles

Altered stresses and dynamics after single and double annuloplasty ring for aortic valve repair.

OBJECTIVESAortic valve repair procedures for the treatment of isolated aortic valve insufficiency may be improved by stabilizing the functional aortic annulus using a double annuloplasty ring at the aortic annulus and sinotubular junction (STJ). The objective of this study was to compare the geometrical changes and aortic root stress distribution when using a single subvalvular ring and a double sub- and supravalvular ring in vivo.METHODSBoth the single- and double-ring procedures were performed successively in nine 80-kg pigs. Measurements were performed intraoperatively using sonomicrometry crystals in the aortic root to evaluate geometrical changes and annular and STJ force transducers measuring the segmental radial stress distribution.RESULTSThe total force in the STJ was significantly reduced after the double-ring procedure from 1.7 ± 0.6 to 0.04 ± 1.1 N (P = 0.001). The double-ring procedure significantly reduced the STJ area from 234.8 ± 37.6 to 147.5 ± 31.8 mm2 (P = 0.001) and expansibility from 17 ± 6% to 8 ± 3% (P = 0.001). With the single-ring procedure, the STJ shape was circular but became more oval with the double-ring procedure. The double-ring procedure did not affect stress distribution or geometry in the aortic annulus.CONCLUSIONSThe double-ring procedure stabilized the whole aortic root by reducing radial stress distribution in the STJ more efficiently than the single-ring procedure. Both area and expansibility were reduced with the double-ring procedure. These results confirm the importance of addressing the entire functional aortic annulus for optimal aortic valve repair procedures.

Isolated aortic insufficiency valve repair with external ring annuloplasty: a standardized approach.

Aortic valve (AV) insufficiency with normal root and ascending aorta is most frequently treated by valve replacement with significant prosthetic-related complications and reduced life expectancy. We compared the outcomes of a new standardized isolated AV repair approach using an external aortic annuloplasty ring at the subvalvular level (single ring annuloplasty) and the role of an additional supravalvular ring at the sinotubular junction (double ring annuloplasty). Single centre data were collected from the Aortic Valve Insufficiency and ascending aorta Aneurysm InternATiOnal Registry (AVIATOR) between 2003 and 2017. A total of 93 patients (56 single ring and 37 double ring) underwent isolated AV repair. The overall 30-day mortality rate and the need for a permanent pacemaker were 1% and 2%, respectively. The overall survival rate at 6 years was similar for sex- and age-matched members of the general population (89% vs 95%; P = 0.1) and did not differ between the double and single ring groups (82% vs 93%; P = 0.4) at 6 years. There were no thromboembolic or bleeding events in the entire cohort. However, at 6 years, the cumulative incidence of valve-related reintervention was 26% in the single ring annuloplasty group compared to 3% in the double ring annuloplasty (P = 0.02) group. Similarly, at 6 years, the cumulative incidence of moderate-to-severe (>2) aortic insufficiency was 30% in the single ring annuloplasty group compared to 0% in the double ring annuloplasty group (P = 0.007). Standardized AV repair with external ring annuloplasty has a survival rate similar to that of the general population. The additional stabilization of the sinotubular junction with a second supravalvular ring (double ring annuloplasty) is associated with better outcomes compared to single subvalvular annuloplasty. It can be considered as a first line intervention for patients with isolated aortic insufficiency and pliable leaflets.

Estenose mitral supravalvar em felino de dois anos

Mitral valvular stenosis or supravalvular Mitral stenois (SVMS) is a congenital heart disease rarely diagnosed in cats. It is characterized by narrowing of the valve orifice or by presence of a supravalvular ring causing obstruction to mitral inflow and impaired atrial emptying, leading to increased left atrial pressure and pulmonary edema. Clinical signs include dyspnea, pulmonary hypertension, and arterial thromboembolism due to blood stasis associated with left atrial enlargement. We report the case of a 2-year-old feline with signs of aortic thromboembolism. The presence of supravalvular ring, and turbulent transmitral diastolic flow, confirming the diagnosis of SVMS. The importance of the inclusion of Mitral stenosis in the differential diagnosis of left atrial enlargement and aortic thromboembolism in felines is highlighted.

Shone’s Syndrome and Patent Ductus Arteriosus: A Rare Case Report

Introduction: Shone syndrome is a rare congenital anomaly consisting of multiple levels of obstruction in the left side of the heart and aorta. In its complete form it comprises four types of lesions: (1) parachute mitral valve; (2) supravalvular ring of left atrium; (3) subaortic stenosis of either the muscular or membranous type; or (4) coarctation of the aorta. Shone complex is rarely associated with patent ductus arteriosus. Only two other cases of such association has previously been reported. Case Presentation: We report an 18 year old male referred to our center for aortic and mitral valve replacement who was a near complete case of Shone syndrome associated with a history of repaired coarctation and patent ductus arteriosus during infancy and subsequently repair of aortic valve at 3 years of age. Now he became symptomatic due to the progression of aortic stenosis and congenital mitral valve stenosis. Echocardiography revealed unicuspid aortic valve with severe stenosis and parachute mitral valve deformity with supravalvular ring resulting in severe mitral stenosis. The patient had undergone aortic and mitral valve replacements. Conclusions: This rare case is a near complete form of Shone complex associated with patent ductus arteriosus.It is emphasized that when any form of left heart obstruction is encountered we should meticulously look for other associated lesions.

The “Polar Light Sign” is a useful tool to detect discrete membranous supravalvular mitral stenosis

Mitral valve stenosis caused by a discrete supravalvular membrane is a rare congenital malformation haemodynamically leading to significant mitral valve stenosis. When the supravalvular mitral stenosis consists of a discrete supravalvular membrane adherent to the mitral valve, it is usually not clearly detectable by routine echocardiography. We report about the typical echocardiographic finding in three young patients with this rare form of a discrete membranous supravalvular stenosis caused by a membrane adherent to the mitral valve. These cases present a typical echocardiographic feature in colour Doppler generated by the pathognomonic supramitral flow acceleration. Whereas typical supravalvular mitral stenosis caused by cor triatriatum or a clearly visible supravalvular ring is easily detectable by echocardiography, a discrete supravalvular membrane adjacent to the mitral valve leaflets resembling valvular mitral stenosis is difficult to differentiate by routine echocardiography. In our opinion, this colour phenomenon does resemble the visual impression of polar lights in the northern hemisphere; owing to its typical appearance, it may therefore be named as "Polar Light Sign". This phenomenon may help to detect this anatomical entity by echocardiography in time and therefore improve the prognosis for repair.

Adult Patient with Shone's Syndrome and Patent Ductus Arteriosus

Shone's syndrome is a very rare cardiac malformation composed of four obstructive congenital lesions, which include the parachute mitral valve (PMV), supravalvular ring, subaortic stenosis, and coarctation of aorta. Diagnosis requires a comprehensive examination including a carefully performed echocardiography. It is extremely unusual for a patient to remain undiagnosed during adulthood. Here we report a 26-year-old male patient with Shone's syndrome and patent ductus arteriosus (PDA). The patient reported that he had been suffering from exercise intolerance and aggravating dyspnea. Two years ago, he was operated on in another hospital based on the wrong diagnosis of mitral valve prolapse and subaortic membrane. Transthoracic echocardiography revealed the existence of a PMV, which led to severe mitral stenosis through a previously implanted mitral annular ring. Other components of Shone's syndrome, subaortic membrane and aortic coarctation, together with PDA, were also observed. Another operation was suggested, but the patient refused. During the two year follow-up period, in spite of an atrial fibrillation attack and deteriorating dyspnea, the patient still did not want another operation. Once any of the four components of the Shone's complex is detected, clinicians must look for the presence of other lesions.

The added value of real-time three-dimensional echocardiography in the diagnosis of supravalvar mitral ring: case report and review of the literature.

Supravalvular mitral stenosis is a rare condition characterized by an abnormal ridge, with one or two orifices, covering and obstructing the mitral valve. Preoperative identification of the supravalvular ring is the target for obtaining good surgical results. Two-dimensional echocardiogram and transesophageal echocardiography both failed in reaching this objective. In this case, we showed that three-dimensional echocardiogram is a new technique that provides additional and more accurate echocardiographic characterization of congenital supravalvular mitral stenosis.

A Hammock in the Left Atrium

Shone's anomaly describes a complex involving multiple left sided cardiac obstructions, namely, parachute deformity of the mitral valve, supravalvular ring of the left atrium, subaortic stenosis and aortic coarctation. We are reporting a case of Shone's anomaly characterised by aortic recoarctation, mitral supravalvular membrane, bicuspid aortic valve, complicated complete atrioventricular block and bradycardia-induced nonsustained polymorphic ventricular tachycardia. We revealed mitral supravalvular membrane by 3D transoesophageal echocardiography.

Shone's syndrome diagnosed with echocardiography and confirmed at pathology

The Shone's complex, defined by four cardiovascular defects such as a supravalvular mitral membrane, valvular mitral stenosis by a parachute mitral valve, subaortic stenosis, and aortic coarctation, is a rare entity, which occurs most frequently in its incomplete form. We report the case of a 19-year-old female patient who presented at the emergency room for progressively worsening dyspnoea, orthopnoea, fever, and productive cough, due to bronchopneumonia. Echocardiography revealed the co-existence of aortic coarctation with bicuspid aortic valves, mitral supravalvular ring, and dysplastic mitral valves producing severe mitral stenosis and severe pulmonary hypertension. Although wide spectrum antibiotics were administered from the first day of hospitalization, the patient developed severe sepsis and died. The components of the Shone's complex diagnosed by echocardiography were confirmed at pathology.

An unusual case of tricuspid lesion in congenital corrected transposition of the great arteries

An unusual case of severe supravalvular stenosing ring of the left atrium associated with tricuspid valve dysplasia in an adult symptomatic patient affected by congenitally corrected transposition and unrestrictive ventricular septal defect is reported. The stenosis of the systemic atrioventricular valve possibly prevented the development of obstructive pulmonary vascular disease; removal of the membrane, attached to the tricuspid annulus, together with pulmonary banding was carried out. Clinical conditions improved after surgery. The unusual stenotic lesion of the systemic right ventricular inflow allowed conservative surgical palliation in this adult patient.

Surgical treatment of congenital mitral stenosis: medium-term results

OBJECTIVE: For a cohort of patients with congenital mitral stenosis (CMS), to determine: patient outcomes, predictors of valve repairability and predictors of durability of valve repair. METHODS: From 1989 and 2002, 23 patients underwent surgical treatment of CMS, excluding those with common atrioventricular canal, and univentricular forms. The median age at operation was 15.5 months (range 2-204), and the median body weight was 11 Kg (range 4.5-51.6). Seventeen patients (73.9%) had associated anomalies, including Shone's complex in nine (39.1%) and pulmonary hypertension in 14 (60.9%). Mitral stenosis was severe in 14 patients (60.9%) and moderate in the remaining (median trans-mitral gradient of 16 mmHg, range 8.5-32). Mitral valve repair was performed in 18 patients (78.3%), and valve replacement in five (21.7%). Repair techniques included papillary muscle splitting (n=10), excision of supravalvular ring (n=9) and commissurotomy (n=8). Twelve patients (52.2%) required associated procedures. RESULTS: There were no early and late deaths at a mean follow-up of 58.5 ± 46.7 months (range 1-156). Mean hospital stay was 12.7 ± 8.2 days. There were no significant factors associated with unsuccessful valve repair. Actuarial freedom from reoperation at five years was 67.1% (CI 95%: 56.8% to 77.4%). The mitral valve repair group required reoperation in eight patients (44.4%) (two early and six late), as opposed to one (20%) in the replacement group. The presence of preoperative pulmonary hypertension was significantly related (p<0.005) to higher reoperation rates. All but two the followed patients are presently in functional class I and the echocardiography has shown less than 2+ mitral stenosis and/or regurgitation. CONCLUSION: Reoperations were the most important cause of morbidity at the medium-term follow-up of CMS. Preoperative pulmonary hypertension may predict the need for reoperation after mitral valve repair, which is the procedure of choice in CMS.

Shone's anomaly complicated by ascending aortic aneurysm in a pregnant woman

SHONE ET AL1 described a complex of multiple congenital obstructions involving mitral valve stenosis, supravalvular ring in the left atrium, subaortic stenosis, and coarctation of the aorta. In the incomplete form, some of the obstructions are present, whereas others are absent or only of minor relevance.1,2 A case of Shone's anomaly complicated by an ascending aortic aneurysm in a pregnant woman is described.

A Form Fruste of Shone's Complex Diagnosed by Transesophageal Echocardiography.

Shone's complex is a developmental complex of parachute mitral valve, supravalvular ring of the left atrium, subaortic stenosis, and coarctation of the aorta. Little clinical experience has been accumulated since the original description by Shone et al. This may be due to underrecognition of the condition by transthoracic echocardiography (TTE). We describe a forme fruste of Shone's complex which is definitively diagnosed only after precise demonstration of the anatomy by transesophageal echocardiography (TEE). Demonstration of these abnormalities by TEE was of little clinical significance in our patient, but may be important in others in whom prior TTE has been suboptimal. (ECHOCARDIOGRAPHY, Volume 13, March 1996)

Congenital total absence of the pericardium: Case report of a 72-year-old man and review of the literature

Fig. 1. General appearance of the malformation in the frontal view. 1, Superior vena cava; 2, inferior vena cava; 3, left superior vena cava; 4, malaligned atrial septum and ostium primum defect. restrictive and accounts for elevation of lefl atrial pressure. In aceordance with Carpentier and colleagues, 5 when the papillary muscles are basically normal, congenital mitral valve stenosis may be due to papillary muscle commissure fusion, excess valvular tissue, hypoplasia of the anulus, or supravalvular ring. In this case, the supravalvular ring was incomplete, and most of the stenosis was due to the overriding restrictive ostium primum.

Double-outlet right atrium with restrictive ostium primum and incomplete supravalvular ring presenting as congenital mitral valve stenosis

We have recently encountered a rare case of partial atrioventricular (AV) canal. The malformation included a persistent left superior vena cava draining into a dilated coronary sinus. The left and right auriculoventricular valves were abnormal, as usually seen in partial AV canal. The restrictive ostium primum and surrounding atrial septum were malaligned and displaced in a left posterior position. An incomplete supravalvular ring was found above the lateral component of the left AV valve. This anomaly, which is identified as double-outlet right atrium, was misdiagnosed before the operation and was responsibie for a clinical presentation similar to congenital mitral valve stenosis resulting from supravalvular ring. We present a case to draw attention to this rare diagnosis (double-outlet right atrium) as an unusual cause of impediment to left AV valve inflow and to include it in the differential diagnosis of mitral valve stenosis with basically normal subvalvular apparatus. The patient was a 14-year-old girl, known since childhood to have a cardiac murmur. Although she had reduced physical activity, she developed satisfactorily, without cyanosis. When she was 8 years old, a diagnosis of congenital mitral stenosis resulting from a supravalvular ring was made, and it was confirmed 2 years later by a different pediatric cardiologist. In June 1993, surgical repair was advised owing to cardiac enlargement. The New York Heart Association functional class was II. On physical examination, the patient was a thin prepubertal noncyanotic girl with mild psychomotor impairment. The sternum was of pectus excavatum type. The jugular venous pressure was within normal limits. No peripheral edema was apparent. Cardiac auscultation revealed a holosystolic grade 3/6 murmur, best heard at the apex and the axillar area. There was also a grade 2/6 diastolic murmur. The second heart sound was normal. The lungs were clear to auscultation, and the liver and spleen were not enlarged. The electrocardiogram showed normal sinus rhythm with electrical signs of right ventricular hypertrophy. The cardiothoracic ratio was 0.55, and the aspect of the left ventricle was suggestive of mitral stenosis. The preoperative echocardiogram showed a partial AV canal type

Supravalvular tricuspid ring and Ebstein's anomaly in congenitally corrected transposition

Congenitally corrected transposition of the great arteries (i.e., discordant atrioventricular connections associated with discordant ventriculoarterial connections) is frequently combined with anomalies of the tricuspid valve. 1 Most often, these malformations resemble a form of Ebstein's anomaly, whereas a stenosis or hypoplasia of the tricuspid valve is rare, and a supravalvular ring represents an exceptional event. 1,2 Other malformations associated with congenitally corrected transposition consist of ventricular septal defects followed by abnormalities of the pulmonary outflow tract. 1

Supravalvular aortic stenosis: Repair by extended aortoplasty

Enlargement of the aorta with a diamond-shaped patch of the noncoronary sinus of Valsalva may not be sufficient in severe cases of supravalvular aortic stenosis. This traditional reconstruction is asymmetric, and, if the fibrous supravalvular ring is thick and rigid, the aorta may not open wide with patch angioplasty, so that aortic obstruction may remain. Also, because aortic valve function may not be perfect after asymmetric reconstruction, there may be aortic valve incompetence or obstruction of coronary ostia by the valve cusps. A new reconstructive operation was designed and used in eight patients. All survived and are asymptomatic. The aortoplasty was extended so that the supravalvular ring was incised at two points in the noncoronary and in the right coronary sinuses of Valsalva. The area of stenosis was opened wide, and the cusps of the aortic valve were lengthened, which provided better approximation and function. A tubular Dacron prosthesis, tailored to reconstruct the aorta, provided a wide aortic cross-sectional area. This technique of extended aortoplasty for symmetric reconstruction of the aorta should provide more predictable relief of aortic obstruction and improved function of the aortic valve.

Supravalvular stenotic mitral ring: A new case with surgical correction

In this report, we present a case of supravalvular stenotic mitral ring in a child. The lesion created a picture of severe mitral stenosis and was successfully managed by resection of the ring. No other malformations of the mitral apparatus or left ventricular outlfow tract were found. On analyzing the diagnostic criteria, we concluded that there appears to be only one useful criterion--the angiographic morphology. However, when ther- is an associated ventricular septal defect or findings of Shone's complex, we should suspect supravalvular ring rather than any other form of mitral stenosis.

Congenital heart malformations associated with disproportionate ventricular septal thickening.

Asymmetric septal hypertrophy, or ASH, is a genetically determined myocardial disorder that is transmitted as an autosomal dominant trait. ASH is characterized by a disproportionately thickened ventricular septum that contains numerous hypertrophied, bizarrely-shaped and disorganized cardiac muscle cells. Disproportionate hypertrophy of the ventricular septum has also been observed in association with certain congenital cardiac malformations. To determine whether such congenital cardiac malformations are part of the disease spectrum of genetically determined ASH, cardiac pathologic observations were made in eight patients with disproportionate septal thickening (ventricular septal to posterobasal left ventricular free wall thickness ratios of 1.5 to 2.5) and the following three categories of associated lesions: 1) parachute deformity of the mitral valve (occurring either as an isolated lesion or with ventricular septal defect, coarctation of the aorta, supravalvular ring of the left atrium, or double outlet right ventricle); 2) complete interruption of the aortic arch; and 3) ventricular septal defect. The arrangement of cardiac muscle cells in the disproportionately thickened ventricular septum was normal in six of the eight patients; in the other two patients (one with parachute deformity of the mitral valve and one with ventricular septal defect) numerous bundles of hypertrophied cardiac muscle cells were interlaced in a disorganized fashion among more normally arranged bundles of cells. First degree relatives of six of the eight patients were studied by echocardiography and found to have normal ventricular wall thicknesses and septal-free wall ratios. It is concluded that disproportionate ventricular septal thickening may occur in patients with a variety of congenital heart malformations, but that such a finding is not necessarily a manifestation of the disease spectrum of genetically determined ASH.

Supravalvular stenosing ring of the left atrium associated with corrected transposition of the great vessels

A case of supravalvular stenosing ring of the left atrium is described. The severity of the stenosis obscured the features of Ebstein's malformation of the left atrioventricular valve, which was the only other defect associated with corrected transposition. Analysis of the reported examples of supravalvular stenosing rings reveals that the condition rarely exists as an isolated anomaly but tends to occur in 2 groups of conditions, namely, a developmental complex of obstructive anomalies of the left side of the heart (supravalvular ring, subaortic stenosis, parachute mitral valve and coarctation of the aorta) or a second group associated with a left to right shunt produced by a ventricular septal defect or patent ductus arteriosus. The potential diagnostic value of echocardiography in cases in which there is evidence of pulmonary venous obstruction is discussed.