Shone's Complex Research Articles

Ascending-to-Descending Aortic Bypass: A Simple Solution to a Complex Problem

Surgical correction of complex aortic coarctation can be associated with significant risks. Extraanatomic bypass may represent a safer alternative. Between January 1985 and December 2012, 80 consecutive patients with complex coarctation underwent ascending-to-descending aortic bypass through a median sternotomy. Patients were a median age of 42 years (range, 15 to 75 years), and 51 (64%) were males. Recurrent coarctation was present in 52 patients (65%), with 6 (8%) having undergone balloon dilatation. Uncontrolled hypertension was present in 63 patients (79%). The most common concomitant pathology was aortic valve stenosis in 21 patients (26%), subaortic stenosis in 10 (13%), and Shone complex in 4 (5%). There were no early deaths. The most common concomitant procedures were aortic valve replacement, coronary artery bypass grafting, and resection of subaortic stenosis. The mean aortic cross-clamp and cardiopulmonary bypass times were 33 ± 40 and 106 ± 54 minutes, respectively. Morbidity included atrial fibrillation in 17 patients (21%) and reexploration for bleeding in 6 (8%). There was no paraplegia or stroke. Upper extremity blood pressure significantly improved (p < 0.001). Mean systolic blood pressure decreased from 153 ± 26 mm Hg preoperatively to 123 ± 15 mm Hg postoperatively. Mean follow-up was 7 ± 6 years (maximum, 22 years). Late deaths occurred in 5 patients (6%) and were not graft-related. Three patients (4%) required reoperation for repair of periprosthetic regurgitation in 2 and mitral valve replacement in1. The ascending-to-descending aortic bypass can be performed with low morbidity and mortality. It is an effective solution to complex aortic coarctation and represents a safe single-stage approach for patients with concomitant cardiac pathology.

Potts Shunt and Atrial Septostomy in Pulmonary Hypertension Caused by Left Ventricular Disease

We report a 20-year-old patient with Shone's complex and severe diastolic dysfunction of his small left ventricle (LV) in whom severe pulmonary hypertension and biventricular failure developed while he was awaiting combined heart-lung transplantation. We performed a percutaneous balloon atrial septostomy and a modified Potts shunt (13-mm graft from left pulmonary artery to descending aorta), with the aim of decompressing the hypertensive right ventricle (RV) by reducing left ventricular preload and left atrial hypertension. The procedures were uneventful. The patient's condition improved rapidly and biventricular function was restored. In contrast to a Potts shunt in other conditions, patients with pulmonary hypertension caused by left ventricular disease may benefit from an additional atrial left-to-right shunt.

Multimodality imaging of an adult with Shone complex

Shone complex is a rare combination of valvular or supravalvular aortic stenosis, supra-valvular mitral membrane, parachute mitral valve, and coarctation of the aorta. This article presents an unusual case of a 40-year-old woman who presented with a history of progressive dyspnea. We describe the main imaging features and protocol used for visualizing this syndrome using various imaging modalities.

Midterm Results of the Modified Ross/Konno Procedure in Neonates and Infants

The management of congenital aortic stenosis in neonates and infants continues to be a surgical challenge. We have performed the modified Ross-Konno procedure for patients who have severe aortic insufficiency or significant residual stenosis after balloon aortic dilation. The midterm results of this procedure were evaluated in this subset of patients. Between 1994 and 2010, a total of 24 patients younger than 1 year of age underwent the modified Ross-Konno procedure. The diagnoses were aortic stenosis with or without subaortic stenosis (n = 16), Shone's complex (n = 7), and interrupted aortic arch with subaortic stenosis (n = 1). The aortic root was replaced with a pulmonary autograft, and the left ventricular outflow tract (LVOT) was enlarged with a right ventricular infundibular free wall muscular extension harvested with the autograft. Age at operation ranged from 1 to 236 days (median 28 days). The median follow-up period was 81 months (range 1-173 months). There was 1 early death and no late mortality. Overall the 1-, 2-, and 5-year survival rate was 95% ± 4.5%. Freedom from aortic stenosis was 94.7% ± 5.1% at 1, 2, and 5 years. Less than mild aortic insufficiency was 93.3% ± 6.4% at 2 years, and 74.7% ± 12.9% at 5 years. In total, 23 reoperations and reinterventions were performed; 14 were allograft conduit replacements. Two patients required aortic valve plasty. None required valve replacement. The reintervention-free rate was 64.6% ± 10.8% at 2 years and 36.9% ± 11.3% at 5 years. Pulmonary autografts demonstrated good durability with low mortality and morbidity. This study shows that the modified Ross-Konno procedure can be a practical choice in selective cases for complex LVOT stenosis in neonates and infants.

Foetal echocardiographic assessment of borderline small left ventricles can predict the need for postnatal intervention

We sought to prospectively determine foetal echocardiographic factors associated with neonatal interventions in borderline hypoplastic left ventricles. Foetuses were included who had a left ventricle that was 2-4 standard deviations below normal for length or diameter and had forward flow across the mitral and aortic valves. Factors associated with an intervention in the first month of life or no need for intervention were sought using univariate and multivariate logistic regression models. From 2005 to 2008, 47 foetuses meeting the criteria had an additional diagnosis (+foetal coarctation/+transverse arch hypoplasia): atrioventricular septal defect 7 (+2/+0), double outlet right ventricle 2 (+0/+0), Shone's complex 19 (+9/+4), and ventricular disproportion 19 (+13/+11; 4 both). There were seven pregnancies terminated, three foetal demises, and five had compassionate care. There were 32 livebirths that either had a biventricular repair (n = 20, n = 2 dead), univentricular palliation (n = 2, both alive), or no intervention (n = 9). Overall survival of livebirths to 6 months of age was 79%. Factors associated with early intervention on first foetal echocardiogram were: obstructed or retrograde arch flow (p = 0.08, odds ratio 3.3), coarctation (p = 0.05, odds ratio 11.4), and left ventricle outflow obstruction (p = 0.05, odds ratio 12.5). Neonatal factors included: Shone's diagnosis (p = 0.02, odds ratio 4.9), bicuspid aortic valve (p = 0.005, odds ratio 11.7), and larger tricuspid valve z-score (p = 0.05, odds ratio 3.6). A neonatal factor associated with no intervention was a larger mitral valve z-score (mean 23.8 versus 24.2 intervention group, p = 0.04, odds ratio 2.8). The need for early intervention in foetuses with borderline hypoplastic left ventricle can be predicted by foetal echocardiography.

Shone’s complex with dextrocardia and situs inversus totalis: a case report

Parachute mitral valve complex is an unusual congenital anomaly that has been described by Shone et al. It is characterized by a parachute deformity of the mitral valve associated with additional forms of left heart anomalies, such as aortic valvular stenosis and coarctation of the aorta. A 21-year-old female who was referred to our department because of progressive dyspnea on effort and at rest and minimal cyanosis is presented in this case report. On cardiac auscultation, the patient had a grade III/VI pansystolic murmur best heard at the lower left sternal border. The chest X-ray demonstrated dextrocardia and mild cardiomegaly. Echocardiographic evaluation revealed Shone's complex, including parachute mitral valve anomaly.

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.

Abstract 18187: Shone's Complex: Single Center Experience Over 22 Years

Background: Children with Shone's Complex (SC) have multiple levels of left heart obstruction and often require a number of left heart interventions. They are at risk of long-term morbidity and mortality. However, long-term outcomes in children with SC are limited. Methods: SC was defined as a mitral valve (MV) anomaly and ≥ 1 other left heart obstructive lesion including coarctation of the aorta (CoA), subvalvar, supravalvar and aortic valvar stenosis (AS). MV morphology type was classified as typical (MVtyp) - hypoplastic annulus, thickened leaflets; mitral ring (MVr); arcade or other papillary muscle anomaly (MVarc); parachute (MVpap); and other including double-orifice (MVo). Pts who underwent Norwood palliation were excluded. We reviewed overall outcomes and focused on the impact of pulmonary hypertension (PH) and pre-existing atrial septal defect (ASD) on left heart growth, number of interventions, and death/transplant (OHT). Results: A total of 129 SC pts presented at 35d (0d - 18yrs) of age and were followed for 7.0 ± 6.8 years. MV morphology included MVtyp (n=89, 68%), MVr (n=23, 18%), MVpap (n=21, 16%), MVarc (n=25, 19%). A total of 113 pts underwent 232 (median 2, 0-5) interventions including ASD closure (n=49), VSD closure (n=30), AVR (n=8), MVR (n=10), MV repair (n=39), AS repair (n=37), balloon valvuloplasty (n=36) and CoA dilation/repair (n=60). Pre-intervention, ASD was associated with lower mean LA pressure 11.7±5.6 vs 18.5±6.5 mmHg (p=0.02) and lower mitral gradient 5.3 ± 5.6 vs 10.9 ± 10.6 mmHg (p&lt;0.01) on catheterization. Following first surgical intervention, residual ASD was associated with decreased annular growth of AV (p=0.042) and MV (p=0.01), smaller increase in LVEDD (p=0.001) and LVESD (p=0.018). MVpap was associated with smaller MV (p&lt;0.001) and less MV annular growth by latest follow-up (p=0.001). Twenty-year OHT-free survival was 87%. By multivariate analysis, PH was associated with death/OHT (OR 6.8, 1.7-27.8). Survival in pts without residual ASD was 88% vs 77% with an ASD (p=0.017). Conclusions: SC pts with residual ASD have a higher rate of interventions. Left heart growth is optimized with ASD closure, while MVpap is associated with decreased growth. Pts who develop PH and have residual ASDs are at higher risk of mortality.

The Dilemma of Subaortic Stenosis – A Single Center Experience of 15 Years with a Review of the Literature

The term "subaortic stenosis" includes a variety of obstructions of the left ventricular outflow tract (LVOT), ranging from a short (discrete) subvalvular membrane to long, tunnel-like narrowing. An association with other congenital lesions is frequent. We reviewed the reported literature and describe our results, analyzing the nomenclature of and risk factors for restenosis after surgical treatment. From 1994 to 2009, 81 children (53 males, 28 females; median age: 57 months, range [ R]: 5-204) underwent surgical relief of a subaortic stenosis. Patients were divided, according to pathology, into short segment (group A, n = 42) and complex obstructions (group B, n = 39), with the latter including long segment stenosis and/or associated anomalies such as aortic coarctation, interrupted aortic arch or Shone's complex. Surgery resulted in a significant reduction of the gradient between the left ventricle and the aorta in both groups (Δ P group A: 51 ± 28 mmHg, group B: 46 ± 25 mmHg). There was no operative mortality. One patient died in the early postoperative period due to pericardial tamponade. Median follow-up was 90 months (R = 0.5-187). Twenty-five (31%) patients required reoperation because of recurrent stenosis after a median of 43 months (R = 0.5-128). Seven (16%) patients belonging to group A developed restenosis, and 18 (46%) in group B. Freedom from reoperation for all patients was 60% after 10 years. 10 (40%) of the patients of group B were ultimately treated with a Ross-Konno reconstruction of the LVOT. Despite adequate surgical resection, recurrence of subaortic stenosis within several years after initial surgical treatment is frequent, especially in patients with complex lesions. In cases requiring reoperation, the surgical therapy is often extensive, and even includes Ross-Konno reconstruction of the LVOT.

Atypical Shone’s Complex Diagnosed by Echocardiography

Shone's complex is a rare and severe type of congenital left-sided obstructive lesion of multiple cardiovascular levels. This report aims to present the authors' experience diagnosing atypical Shone's complex using echocardiography. Atypical Shone's complex was diagnosed for 38 consecutive patients (18 boys and 20 girls, ages 1 day to 15 years) using echocardiography. All the diagnoses were confirmed by cardioangiography, surgery, or both. Among the congenital left-sided obstructive lesions, four levels of obstruction coexisted in 3 cases (7.9%), three levels in 10 cases (26.3%), and two levels in 25 cases (65.8%). Supra-annular mitral stenosis caused by a supravalvular mitral ring was diagnosed in 1 case. Mitral valvular stenosis was diagnosed in 24 cases including a parachute mitral valve (4 cases), a double-orifice mitral valve (1 case), and partly fused thickened mitral valvular leaflets (19 cases). Subaortic stenosis was diagnosed in 10 cases including circumferential membranous subaortic stenosis (1 case), tunnel-type subaortic narrowing (2 cases), and asymmetric obstructive fibrous ridge below the aortic valve (7 cases). Aortic stenosis was diagnosed in 25 cases including the bicuspid aortic valve (12 cases), the hypoplasia aortic annulus (3 cases), and partly fused or thickened tri-leaflet aortic valves (10 cases). Supra-aortic stenosis was diagnosed in 6 cases including the localized type (4 cases) and the diffused type (2 cases). Coarctation of the aorta was diagnosed in 26 cases. Of these, focal coarctation was described in 15 cases, long segment coarctation in 7 cases, and dysplastic isthmus and/or descending aorta in 4 cases. Secondary changes in Shone's complex manifested as left atrial dilation (26 cases), left ventricular hypertrophy (26 cases), and pulmonary hypertension (19 cases). Echocardiography plays an important role in the diagnosis of atypical Shone's complex, in comprehensive evaluation of the number, position, morphology, and severity of left-sided obstructions.

Multi-Modality Imaging of an Adult Parachute Mitral Valve

A parachute abnormality of the mitral valve is an extremely rare finding in adults. It is usually seen as part of Shone's complex. The authors present multimodality imaging from a case of adult parachute abnormality of the mitral valve to illustrate and explain features such as the characteristic "pear" shape of the valve and "doming" of the subvalvular apparatus. The solitary papillary muscle that defines the condition may be difficult to identify on transthoracic echocardiography, but redundancy of the chordae is a key echocardiographic feature in the adult form of the condition.

Parachute Mitral Valve in Adults—A Systematic Overview

Parachute mitral valve (PMV) is a rare congenital anomaly of the mitral valve apparatus seen in infants and young children. In most instances PMV is associated with other congenital anomalies of the heart, in particular obstructive lesions of the mitral inflow (mitral valve ring) and left ventricular outflow tract (subaortic stenosis), and coarctation of aorta and is referred to as Shone's complex or Shone's anomaly. PMV may also occur as an isolated lesion or in association with other congenital cardiac anomalies. Not much is known about PMV in adults as an isolated anomaly or in association with other congenital cardiac anomalies. We reviewed the literature to identify cases of PMV (isolated or associated with other lesions) in adults, to address prevalence, clinical presentation, diagnosis, treatment, and outcome of such patients. (Echocardiography 2010;27:581-586).

Double-orifice mitral valve associated with bicuspid aortic valve: a rare case of incomplete form of Shone's complex

Double-orifice mitral valve (DOMV) is a rare congenital malformation characterized by two separate valve orifices of varying sizes in association with the abnormalities of the subvalvular apparatus. It has been reported to be associated with a variety of other congenital malformations. We report a rare case of incomplete form of Shone's complex composed of a complete bridge-type DOMV and bicuspid aortic valve, well demonstrated by both transthoracic and transoesophageal echocardiography and cardiac magnetic resonance imaging.

Outcomes of Reparative and Transplantation Strategies for Multilevel Left Heart Obstructions With Mitral Stenosis

Conventional management for multilevel left heart obstructions and mitral stenosis (Shone's complex) involves multiple operations that carry additive risks. This study reviews our experience with reconstructive and transplantation approaches for Shone's complex. Between 1987 and 2007, 43 patients with mitral stenosis and one or more left-sided obstructions were identified: supramitral ring (n = 13), subaortic stenosis (n = 25), aortic stenosis (n = 24), hypoplastic arch (n = 20), and coarctation (n = 38). Thirty patients underwent a staged reparative approach, including 27 mitral and 51 left ventricular outflow tract operations. Thirteen patients were referred for transplantation. Patients with severe hypoplasia of the left ventricle were excluded. There was one in-hospital death (2.5%) and six late deaths (14.2%). Actuarial 5- and 10-year survival for staged surgical and transplantation was 88% vs 61.3% and 83.1% vs 61.3% (p = 0.035). At a mean follow-up of 7.9 years, freedom from mitral reoperation was 83.3% and freedom from reoperation for subaortic stenosis was 78.0%. Wait-list mortality was 13.3% (2 of 13). Wait-list time exceeding 90 days was an incremental risk factor for death after transplantation (p = 0.005). Despite the challenges of a reparative strategy for Shone's complex, favorable survival and durability outcomes can be expected. Heart transplantation, although avoiding the pitfalls of staged repair, confers increased risks from ongoing physiologic derangements due to uncorrected left heart inflow and outflow obstructions during the wait for donor heart availability.

Anomalies of Left Coronary Artery Origin Affecting Surgical Repair of Hypoplastic Left Heart Syndrome and Shone Complex

There has traditionally been less concern regarding coronary anomalies with left-sided congenital heart lesions such as hypoplastic left heart syndrome (HLHS)or Shone complex than with other lesions. However, coronary anomalies in this setting can profoundly affect surgical intervention, particularly when surgical repair involves the ascending aorta. We describe four patients with congenital left-sided heart lesions in which left coronary artery (LCA) anomalies substantially affected intervention and outcome. In the first two cases, the coronary anomalies were not identified prospectively and resulted in surgical injury directly to the coronary or to its surrounding region. In the latter two cases, successful identification of the coronary anomaly preoperatively allowed for modification of surgical technique and/or intervention. We conclude that detailed coronary artery assessment should be part of the routine echocardiographic evaluation of congenital left-sided heart lesions that require surgery.

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.

Surgical Strategies and Outcomes in Patients With Shone Complex: A Retrospective Review

Multilevel obstruction of left-sided heart structures was originally characterized by Shone and colleagues. The formulation of an appropriate operative strategy remains problematic. Surgical outcomes have generally been poor. This review examines operative strategies and mid-term outcomes. A retrospective review was done of 28 patients who presented with the diagnosis of Shone complex at a median age of 14 days, with 46% presenting within the first week of life. At presentation, 5 patients were in cardiovascular collapse, and 10 were at New York Heart Association functional class III or IV. All patients had a structurally abnormal mitral complex. A mean transvalvular pressure gradient of 8 mm or greater existed in 15 patients, 6 had a supraannular mitral ring, and 26 had a coarctation of the aorta. Twelve mitral valve operations were done, with an average time from presentation to operative intervention of 15 months. Two patients were lost to follow-up. Of 26 remaining patients, 2 died after a second operative intervention. The average follow-up of 24 patients was 6.3 years (range, 1 to 16 years). Overall survival was 93%. All surviving patients are in class I or II congestive heart failure. Long-term survival of patients diagnosed with Shone complex is excellent. Operative strategies for this complex group should be individualized. Mitral interventions may generally be deferred.

Systemic Sarcoidosis After Cardiac Transplantation in a 9-year-old Child

Sarcoidosis is a granulomatous disease of unknown etiology and is only rarely seen in infants and children. We present the case of a 9-year-old boy who developed sarcoidosis with multi-organ involvement 9 years after cardiac transplantation for Shone complex. The patient was on immunosuppressive therapy with tacrolimus and mycophenolate mofetil. He presented with severe respiratory distress due to marked mediastinal lymphadenopathy and bilateral pulmonary infiltrates in association with fatigue, low-grade fever, hepatosplenomegaly and generalized lymphadenopathy. Lymph node histology showed non-caseating epitheloid cell granulomas and giant cells. Initialization of therapy with prednisolone resulted in prompt clinical recovery and resolution of all symptoms except for the development of mild pulmonary fibrosis. Tapering of the steroids led to recurrence of mediastinal lymphadenopathy 5 months after the initial disease, which responded to an increase in steroid dose. The clinical course, the medical management, and the possible role of immunosuppression in the etiology of the disease are discussed.

Heparin-Induced Thrombocytopenia Complicating Support by the Berlin Heart

Since 1992, miniaturized pulsatile air-driven ventricular assist devices (VADs), "Berlin Heart," have been used at many institutions (36 cases in North America in 19 different institutions) for pediatric use. Heparin-induced thrombocytopenia (HIT II) is a significant complication rarely reported in the setting of adult VAD support; no similar report exists concerning pediatric VAD support. We report on a 13-month-old, 8.1 kg girl who required LVAD support for cardiogenic shock of unclear etiology. The patient had a history of multiple surgical repairs for correction of complex congenital heart disease consisting of a series of left heart obstructive lesions (Shone's complex). Despite aggressive ventilatory and inotropic support, the patient continued to deteriorate and subsequently required extracorporeal life support. After 7 days of conventional venoarterial extracorporeal membrane oxygenation, a 10 ml Berlin Heart VAD was implanted. After implantation, the patient developed persistent low-grade fever of unclear etiology, gradual thrombocytopenia, and deterioration of renal function. On postimplant day 10, the pump required replacement because of concerns about an inlet valve thrombus; the explanted device demonstrated a nearly occlusive clot not appreciable from external inspection. Simultaneously, HIT II was diagnosed as a result of hematology workup for persistent thrombocytopenia. We discuss the unique challenges posed by HIT II complicating pediatric VAD support and in relation to the heparin coating of the device.

Results of Aortic Valve-Sparing and Restoration With Autologous Pericardial Leaflet Extensions in Congenital Heart Disease

The purpose of this study is to evaluate the efficacy of aortic valve-sparing repair with glutaraldehyde-treated autologous pericardium in congenital valvular pathology. Sixty-two patients underwent reparative aortic valve surgery from January 1997 through December 2003. The mean age was 25 +/- 20 years (+/- standard deviation) (range, 10 days to 81 years). Fifty percent (31 of 62) were less than 19 years old at operation. The diagnoses included bicuspid aortic valve (39 patients), ventricular septal defect (14 patients), severe aortic stenosis (6 patients), subaortic stenosis (7 patients), bacterial endocarditis (7 patients), neonatal truncus arteriosus (2 patients), Shone's complex (2 patients), transposition complex (1 patient), double-chambered right ventricle (1 patient), and Marfan's syndrome (1 patient). Twelve patients (19 %) had prior sternotomy and cardiac operations. Valve-sparing techniques included pericardial leaflet extensions in 62 patients, creation of one or more pericardial neoaortic sinuses in 8, subcommissuroplasty in 8, pericardial patch of perforated leaflets in 9, Dacron mesh wrap (Boston Scientific, Wayne, NJ) of dilated ascending aorta in 12, and concomitant tricuspid and mitral valve repairs in 3 and 4 patients, respectively. There was one early death (1.6%). There were no late deaths at a mean follow-up of 25 +/- 16 (range, 0.1 to 72.5 months). Six patients required reoperation and prosthetic or homograft replacement for aortic valve incompetence. One out of 6 reoperations required re-repair. The remaining patients are well with a mean aortic regurgitation grade by echocardiography of 1.3 +/- 0.9 (scale, 0 to 4). Aortic valve repair with pericardial leaflet extension is a promising technique for the growing child.