Shone complex prognosis. Patients typically present in childhood requiring .

Shone complex prognosis Petit, Late outcomes in children with Shone’s complex: a single-center, 20-year experience, Cardiology in Shone complex consists of a constellation of left-sided, usually obstructive, cardiac lesions, including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and aortic coarctation. It can be associated with poor long-term prognosis when compared to Simple-Aortic Coarctation (S-CoA). This report Sep 17, 2021 · Shone’s complex is a rare congenital heart disease associated with significant morbidities including atrial flutter, patient-prosthesis mismatch, and aortic dissection and it is important to raise awareness of this rare syndrome for providers and highlight its potential complications. 2 Aslam S, Khairy P, Shohoudi A, et al. 5%) showed favourable outcome (survival rate: 100%) and a 23. In the complete form, four left-sided defects are present [1]: each anomaly participating in the diagnosis of Shone’s complex. Patients typically present in childhood requiring Shone complex is a rare anomaly that may need multiple procedures for correction. Noteworthy, only 2 of the 8 children had all 4 of these lesions, while others had alternative Jan 10, 2025 · 1 Shone JD, Sellers RD, Anderson RC, et al. Generally, When counseling families regarding fetal Shone's syndrome, it is important to emphasize the wide variety of clinical presentations and possible outcomes. Jan 1, 2016 · The prognosis for patients with Shone's anomaly is poor. 1 The initial description of the Shone complex (SC) included coarctation of the aorta, subvalvular aortic stenosis, parachute mitral valve, and supravalvular mitral ring (Figure 1). The objective of this study is to report the outcomes after Shone's complex repair, the growth of mitral and aortic valve and LVOT, and long‐term survival. 2 Manifestations of Lesion in OB Ultrasound Dec 27, 2024 · We performed an isolated orthotopic heart transplant instead of a combined heart and lung transplant in a 25-year-old woman who had undergone 4 previous mitral valve operations for congenital mitral stenosis, parachute mitral valve, and subaortic stenosis (incomplete Shone's complex). 1%) [6]. Seaback, MD David A. The obstructive lesions in Shone’s complex tend to worsen over time , and the degree of mitral valve obstruction is a strong predictor of surgical mortality and long-term morbidity . 1 Patients born with Shone’s complex and MS who undergo biventricular repair often require multiple surgical interventions, a prolonged hospital stay and are at high risk of death. Masneri, DO Jacob H. Background Shone’s complex is a rare congenital heart disease consisting of a Nov 2, 2018 · Shone syndrome is also known as Shone complex, involving several characteristic cardiac abnormalities: coarctation of the aorta, subaortic stenosis, supravalvular mitral ring, and a parachute Oct 1, 2019 · Introduction and importance: Shone complex is a congenital heart defect consisting of four obstructive defects in the left heart: a mitral supravalvular ring, sub-aortic stenosis, parachute mitral Shone syndrome’s prognosis depends on the mitral valve status with or without the presence of pulmonary hypertension and of other associated defects. Operative strategies for this complex group should be individualized. The long-term prognosis for patients with Shone’s Complex depends on the extent of mitral valve disease, the degree to which the left ventricle is hypoplastic, or abnormally small, and the cumulative effects of surgical treatments. In contrast, Bolling et al. ” 4, 5 Several other congenital heart defects are frequently associated with Oct 4, 2022 · Shone syndrome, also known as Shone complex, is a rare syndrome characterised by left-sided, obstructive congenital heart defects. We aimed to assess whether the degree of ventricular disproportion and 2D-speckle-tracking echocardiography (2D-STE) could improve the Nov 7, 2019 · Objectives: Shone’s complex is a rare congenital cardiac lesion affecting the mitral valve and left ventricular outflow tract. Clinical presentation. 87. 6)-year follow-up free from May 28, 2024 · Objective Infants born with multilevel left heart obstructive lesions (Shone’s complex), especially with mitral valve stenosis (MS), often require multiple surgeries and have a guarded prognosis. 4 Classically, the MV morphology Shone's complex is a collection of four left-sided obstructive lesions, first characterized in 1963, which may occur in complete or incomplete forms. To assess operative results and late outcome, we reviewed the records of 30 consecutive patients seen with Shone's anomaly at our Feb 22, 2024 · Shone complex (or Shone syndrome) is a rare congenital disorder, which consists of supravalvular mitral ring, parachute mitral valve (MV), subaortic stenosis, and coarctation of the aorta (CoA). Long-term outcomes following the Ross procedure in neonates and Sep 1, 2020 · In 1963, Shone et al described a complex of multi-level left-sided obstructive lesions in 8 children. 8176-21) Introduction Shone’s complex is a rare congenital anomaly involving a left-sided obstructive lesion of four cardiovascular levels (1). Differentiation between hypoplastic left ventricle and Shone's complex is crucial and may influence the physician's attitude, the presentation of Oct 4, 2022 · Shone syndrome, also known as Shone complex, is a rare syndrome characterized by left-sided, obstructive congenital heart defects. In addition, 10 patients had left ventricular outflow tract obstructions and coarctation of the aorta, 32 had coarctation of the aorta without left ventricular outflow tract obstructions, and 8 had isolated left ventricular outflow tract obstructions. Shone Syndrome (Shone Complex): Symptoms, Diagnosis and Treatment - Symptoma Malta 0% Feb 25, 2023 · Long-term survival of patients diagnosed with Shone complex is excellent. When only two or three of these components are present, the incomplete form of Shone’s syndrome is Shone Syndrome (Shone Complex): Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis. Heart transplantation, although avoiding the pitfalls of staged Feb 15, 2005 · Early echocardiographic studies of 50 patients with Shone's complex were retrospectively examined to identify left-sided cardiac features associated with progressive mitral valve (MV) disease requiring intervention, Objectives Shone’s complex (SC) is characterized by sequential obstructions of left ventricular (LV) inflow and outflow. g. This finding has been confirmed by our study. Studies evaluating the change of mitral and aortic valve z-score Feb 15, 2005 · Early echocardiographic studies of 50 patients with Shone's complex were retrospectively examined to identify left-sided cardiac features associated with progressive mitral valve (MV) disease requiring intervention, as well as mortality. 1,2 Here we discuss how the patient's severe pulmonary Aug 2, 2024 · Objective Shone’s complex comprises of a combination of congenital cardiac anomalies causing obstructions in the left ventricle’s inflow and outflow tracts. Schoeneck, MD Section Editor: Christopher San Miguel, MD Submission history: Submitted April 13, 2023; Revision received July 6, 2023; Accepted June 30, 2023 Electronically published August 9, 2023 Aug 11, 2023 · obstructive, Shone’s complex can present with a wide spectrum of pathophysiology and clin-ical manifestations. 6)-year follow-up free from events. Nicholson, Michael S. Shone syndrome is thought to be very rare, accounting for less than 1% of all congenital heart disease 1. What is Shone syndrome? Shone syndrome is a collection of eight left-sided obstructive heart lesions. casian mother who had class A1 gestational diabetes and pre-eclampsia, conceived via Despite the challenges of a reparative strategy for Shone's complex, favorable survival and durability outcomes can be expected. Shone's complex is a rare congenital heart disease first described by Shone et al. Shone syndrome is thought to be very rare, accounting for less than 1% of all congenital heart disease . Shehatha and Abdulsalam Yaseen Taha and Aram Jamal Mirza}, journal={Journal of the Egyptian Society of Cardio-Thoracic Surgery}, year={2018}, Jan 25, 2012 · Background Shone’s complex is a rare congenital heart disease consisting of a variety of left ventricular inflow and outflow tract lesions. Of all the cases of congenital heart diseases, it makes up only 0. Patients with Shone's syndrome have been described as having an unsatisfactory operative outcome with a mortality rate Nov 1, 2007 · Long-term survival of patients diagnosed with Shone complex is excellent. 3. Epidemiology. Figure 7. Therefore, each patient should be individually evaluated, with diagno- Oct 4, 2022 · Epidemiology. It involves a combination of four specific heart defects that affect the flow of blood through the left side of the heart. When SC involves two or three levels of obstructive lesions, it is called a non-typical Shone’s complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients. Our only death was an infant with Shone’s complex, which itself is a complex disease process with a Objectives Shone’s complex (SC) is characterized by sequential obstructions of left ventricular (LV) inflow and outflow. The overall prognosis of Shone’s complex de-pends on the severity of the associated pathologies and the progressive nature of the restrictive flow pat-tern. 1,2 Here we discuss how the patient's severe pulmonary Feb 22, 2024 · The severity of MS is the most important indicator of mortality and long-term prognosis. A 26-year-old male with history of bicuspid aortic valve and Shone Complex (syndrome)—status post coarctation repair, subaortic resection and Ross-Konno operation—presenting with 3 months of constitutional and respiratory symptoms. Patients with Shone complex (SC Sep 1, 2020 · In 1963, Shone et al described a complex of multi-level left-sided obstructive lesions in 8 children. This increased survival comes with a new set of medical complications that providers need to be aware of. Shone’s complex is a rare cardiac anomaly consisting of four obstructive lesions of the left heart: supramitral membrane/ring; parachute mitral valve; subaortic stenosis; and coarctation of the Sep 1, 2016 · Request PDF | Shone Complex: An Under-recognized Congenital Heart Disease With Substantial Morbidity in Adulthood | Background: Shone complex consists of a constellation of left-sided, usually Jan 1, 2018 · Surgery of Shone complex is expected to yield good outcome if undertaken before the onset of pulmonary hypertension. Shehatha JS, Taha AY, Mirza AJ. 6 percent of all cases of congenital heart disease. Isolated severe Shone complex, or Shone syndrome, is characterized by the simultaneous presence of supravalvular mitral membrane, parachute mitral valve, muscular or membranous subvalvular aortic stenosis, and Shone Syndrome (Shone Complex): Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis. 7% of the patients with congenital heart disease [11] or 0. Sep 4, 2015 · Shone's complex is a rare cardiac anomaly consisting of multiple level of left sided obstructive lesions including supramitral membrane/ring, parachute mitral valve, subaortic stenosis, and Jan 30, 2024 · Long-term survival of patients diagnosed with Shone complex is excellent. Living with Shone's Complex requires ongoing medical care and monitoring. 7% of all congenital heart disease. Establishing a clear definition is one of the principal c PDF | BackGround Shone’s complex is a rare congenital heart disease consisting of a variety of left ventricular inflow and outflow tract lesions. Sep 1, 2020 · In 1963, Shone et al described a complex of multi-level left-sided obstructive lesions in 8 children. We aim to describe the characteristics of this cohort, with emphasis on echocardiographic and hemodynamic features associated with poor outcomes after Five cases of Shone's anomaly presenting at varied age group with different associated anomaly in which intraoperative TEE played a pivotal role in the management are discussed. 5-7 If all four defects are present, the complex is termed “complete,” although the majority of cases have three or fewer defects present and are considered “incomplete. The complex or syndrome consists of 4 congenital The outcomes after Shone's complex repair, the growth of mitral and aortic valve and LVOT, and long‐term survival are reported. Shone Syndrome (Shone Complex): Symptoms, Diagnosis and Treatment - Symptoma Great Britain Objective: Shone's syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. Survival into adulthood is presumed to be due to a PDA, at the expense of a right-to-left shunt. Shone syndrome’s prognosis depends on the mitral valve status with or without the presence of pulmonary hypertension and of other associated defects. Methods An electronic literature search of PubMed and Scopus was performed to identify relevant Despite the challenges of a reparative strategy for Shone's complex, favorable survival and durability outcomes can be expected. Due to a wide array of potential left heart manifestations that may include coarctation of the aorta, subaortic stenosis, supravalve mitral Aug 15, 2015 · Shone’s complex is a severe and rarely seen congenital anomaly. On this page: Article: Epidemiology; Clinical presentation; Treatment and prognosis. 5%), showed a favourable long-term surgical outcome, with the survival rate of 100% and a 23. 6% and the population studied concerns mainly children who receive Aug 12, 2023 · The natural history and prognosis of Shone’s complex are highly variable and dependent on the severity of each of the involved lesions. All surviving patients are in class I or II congestive heart failure. pneumonia), fatigue, water retention, swelling of the extremities, heart murmur, shortness of breath, enlargement of the heart, pulmonary hypertension, and congestive heart failure. Shone and his colleagues in 1963 [1]. Originally described by Shone et al 1 in 1963, complete SC consists of 4 anomalies: supravalvular ring in the left atrium, muscular/membranous type of subaortic stenosis, parachute Jun 1, 2020 · Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension (PH). The syndrome consists of four defects; a supravalvar mitral membrane, parachute mitral valve, subaortic stenosis Aug 6, 2024 · Shone’s complex is rare and represents <1% of all congenital heart disease. Generally, Shone Syndrome (Shone Complex): Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis. Older patients may experience syncope (loss of consciousness), hemoptysis Dec 7, 2020 · The symptoms associated with Shone’s Complex include lack of appetite, coughing, respiratory infections (e. These defects can vary in severity and may include supravalvular mitral membrane, parachute mitral valve, subaortic stenosis, and coarctation of Shone's complex is a series of four obstructive or potentially obstructive left-sided cardiac lesions (supravalvular mitral ring, parachute deformity of the mitral value, subaortic stenosis, and Nov 1, 2004 · An incomplete form has been described when only two or three of the components are recognized. DOI: 10. On one end of the spectrum in the case of a mild degree of obstruction of one or two isolated lesions, there may be no clinical sequelae, and no interventions are needed; however, on the other severe end, the Work-up revealed multiple congenital defects, reminiscent of the Shone’s complex. Request PDF | Multiple left-sided stenotic lesions: Outcomes after mitral valve surgery. The prognosis can vary widely based on the severity of Feb 4, 2025 · What is Shone's complex? Shone’s syndrome is a rare condition, even among congenital heart defects. The overall prognosis of Shone’s complex de-pends on the severity of the associated pathologies . Neonatal mitral valve stenosis (MS) is a rare congenital heart disease and can be associated with a spectrum of left sided heart and great vessel hypoplasia and/or obstructions known as Shone’s complex. There are a few studies evaluating the long-term outcomes in this population. Partial forms of Shone complex are rare. To assess operative results and late outc Mar 1, 2018 · Long-term survival of patients diagnosed with Shone complex is excellent. De la Uz, Ricardo H. The prognosis for patients with Shone's anomaly is poor. Surgical outcomes of the complete forms have generally been poor, whereas there is a lack of data on long-term follow-up of surgically treated adult partial complex. There are a few studies evaluating the long-term outcomes in this Long-term survival of patients diagnosed with Shone complex is excellent. Clinical presentation and prognosis of patients with this anomaly are dependent on the complexity and severity of the different obstructive lesions. Patients typically present in childhood requiring early surgical intervention; however, with improved surgical techniques, these patients are surviving later into adulthood. This systematic review aims to evaluate the clinical features and surgical outcomes of Shone’s complex. Kaplan–Meier survival curve of 121 patients with Shone complex treated over a 32-year period demonstrating transplant-free 10- year survival of 86%. Older patients may experience syncope (loss of consciousness), hemoptysis Jul 26, 2024 · Shone`s Complex is a well-known structure in the field of artificial intelligence. There is a paucity of data regarding long Shone complex is defined by 4 anomalies: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and coarctation of the aorta. 1 While at least 4 lesions are required to diagnose complete Shone complex, partial Shone complex has been described when patients have only 2 or 3 of these left-sided Incomplete Shone's complex in the sixth decade of life: echo and cardiac magnetic resonance imaging assessment 38) of the 38 cases of partial forms of Shone’s complex in-cluded in their review (2). Surgical intervention is required in all cases. On one end of the spectrum in the case of a mild degree of obstruction of one or two isolated lesions, there may be no clinical sequelae, and no interventions are needed; however, on the other severe end, the Jan 1, 2025 · Shone complex (SC) is a rare multilevel congenital heart disease (CHD) involving four levels of obstructive lesions of the left-side of the heart: beginning with a supravalvular mitral ring, followed by parachute mitral valve (PMV), subaortic stenosis, and coarctation of the aorta (CoA) [1]. and mild or moderate aortic insufficiency were associated with a poor prognosis. We report the case of a 57-day-old boy transferred to pediatric department for persistent dyspnea. (1,2,4,5) Most of the patients have severe Key words: Shone’s complex, forme fruste of Shone’s complex, double-orifice mitral valve, bicuspid aortic valve, aortic coarctation (Intern Med 61: 1367-1370, 2022) (DOI: 10. 2018. It is a congenital heart disease, consisting of multiple levels of left sided obstructive lesions including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation of aorta. 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 Request PDF | Prenatal diagnosis of Shone's syndrome: Parental counseling and clinical outcome | To describe a series of fetuses diagnosed as having Shone's syndrome, which includes four cardiac It is estimated that Shone complex comprises 0. Heart transplantation, although avoiding the pitfalls of staged Oct 4, 2022 · Shone syndrome, also known as Shone complex, is a rare syndrome characterized by left-sided, obstructive congenital heart defects. 2022; 3(6): 1892. Since its first description by Shone et al. The prognosis of the treated patients depends on 2 factors; the severity of MV obstruction [7] and the timing of surgery. , in 1963, literature comes from isolated case reports mainly in children. 3 years (range, 1 to 16 years). It accounts for approximately 0. 6–0. Jan 4, 2019 · Shone’s complex (or syndrome) is a rare congenital heart condition characterized by multiple left heart defects. Noteworthy, only 2 of the 8 children had all 4 of these lesions, while others had alternative Feb 23, 2022 · Background Shone’s complex is a rare congenital heart disease consisting of a variety of left ventricular inflow and outflow tract lesions. 4 Classically, the MV morphology Jul 26, 2016 · Shone’s syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. It mainly comprises of a set of four left-sided cardiac defects, namely: supravalvular mitral membrane (SVMM), parachute mitral valve, subaortic stenosis (membranous or muscular) and coarctation of the aorta. Shone’s complex represent about 0. The preoperative echocardiographic findings were examined against the intraoperative findings. The prognosis of the treated patients depends on 2 factors; the severity of MV obstruction and Surgery of Shone complex is expected to produce good outcome if performed before the beginning of pulmonary hypertension. The severity of the symptoms and the prognosis Shone complex is a rare congenital cardiac malformation characterized by a complex of four obstructive lesions of the left heart: supravalvular mitral membrane, parachute mitral valve, muscular or membranous subvalvular aortic stenosis and coarctation of aorta. View Show abstract Jan 1, 2024 · Shone’s complex (SC) represents a constellation of congenital anomalies characterized by the presence of multilevel obstruction in the left-sided circulation. On clinical examination, patients may have murmurs heard over both the aortic and mitral areas during precordial Mar 15, 2022 · Overall survival was 93%. 1016/J. It is a fascinating research area that studies the phenomenon whereby an AI system`s performance improves in particular tasks as more data is fed to it. Sixty-six patients were included. We report on an 18 year-old woman with a Clinical presentation and prognosis of patients with this anomaly are dependent on the complexity Oct 10, 2023 · The main survival is 75% in a 15-year follow up. Multilevel obstruction of left-sided heart structures was originally characterized by Shone et al. Between 2001 and 2011, nine Enter the email address you signed up with and we'll email you a reset link. Increased Nov 1, 2021 · The severity of mitral valve obstruction still the main predictor of prognosis [1, 2], the long-term prognosis with this complex is poor, with a mortality rate of 24%–27% [4]. The diagnosis of atypical Shone’s complex with three levels of obstruction was made. Shone's complex is a series of four Enter the email address you signed up with and we'll email you a reset link. Patients, usually neonates and infants, present with signs and symptoms of congestive cardiac failure . Incomplete Shone complex consists of a mitral valve anomaly associated with lesions involving the subaortic region, aortic valve, or thoracic aorta. Shone Syndrome is a rare congenital heart condition characterized by multiple left-sided heart obstructions. Oct 10, 2023 · Shone’s syndrome (SS)heart is a rare congenital disease described by Shone in 1963. Arguments for abandoning the eponym ‘Shone syndrome’ | Objectives: Management of patients with left Jan 1, 2022 · Patients surgically treated before the age of 5 (87. The long-term prognosis is poor, and the perioperative mortality rates are 24%-27% [13, 14]. 03. Apr 1, 2010 · Long-term survival of patients diagnosed with Shone complex is excellent. Jul 18, 2022 · The prognosis of this anomaly depends on the complexity and the severity of the different obstructive lesions [1,5,6]. 1 The initial description of the Shone complex (SC) included coarctation of the aorta, subvalvular aortic stenosis, parachute mitral valve, and supravalvular mitral ring (Figure 1). In Shone’s complex, the overlap of multiple obstructive lesions makes it difficult to assess the severity of each ob-structive lesion, and mitral stenosis is typically associated with a poor prognosis among obstructive lesions (12, 13). Am J Cardiol 1963;11:714–25. Shone Syndrome (Shone Complex): Symptoms, Diagnosis and Treatment - Symptoma South Africa Shone Syndrome (Shone Complex): Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis. 16 Shone complex is a rare congenital cardiac malformation characterized by a complex of four obstructive lesions of the left heart: supravalvular mitral membrane, parachute mitral valve, Feb 20, 2025 · Prognosis and Long-term Management. The multiple sites of blockage in the blood flow through the left side of the heart differentiates Shone’s syndrome from other isolated heart defects. J Clin Images Med Case Rep. References. We aimed to assess Oct 21, 2022 · The overall prognosis of Shone’s complex de-pends on the severity of the associated pathologies and the progressive nature of the restrictive flow pat-tern. Pignatelli, Nancy A. Shone syndrome was identified by Dr. Since then, several Shone’s complex descriptions with three or two levels of obstruction have been reported and called incomplete Shone’s complex or atypical. Sep 16, 2021 · Objective: Shone's syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. The severity of the condition is dependent on the complexity of the individual lesions involved, 2 and the prognosis of Shone complex depends on the degree of obstruction across Shone's complex is a series of four obstructive or potentially obstructive left-sided cardiac lesions (supravalvular mitral ring, parachute deformity of the mitral value, subaortic stenosis, and Shone's anomaly, a congenital cardiac malformation, consists of multiple levels of left heart obstruction including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation. Prognosis; Prevention; Symptoms of Shone`s Complex. | Find, read and cite all the research you Shone's complex is a rare cardiac anomaly consisting of four obstructive lesions of the left heart: supramitral membrane/ring; parachute mitral valve; subaortic stenosis; and coarctation of the aorta. In patients Jul 18, 2022 · Survival in patients with SA is now favorable. Conclusions: Long-term survival of patients diagnosed with Shone complex is excellent. These affect blood flow to and from the left ventricle, or lower left heart chamber. This report Oct 25, 2024 · Long-term survival of patients diagnosed with Shone complex is excellent. A good outcome in anticipated in those operated upon prior to Shone's Complex and the Adult Patient. We sought to determine the long-term outcomes in our paediatric population with Shone’s syndrome and the factors associated with left heart growth. Shone's complex is a rare lesion affecting the mitral valve (MV) and left ventricular outflow tract (LVOT). He described four lesions Feb 1, 2017 · Shone complex may also be referred to as Shone anomaly and Shone syndrome. Its incidence is o. Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the May 2, 2022 · complete Shone complex out of 28 cases (7. a second operative intervention. When only two or three of these components are present, the incomplete form Feb 1, 2024 · Long-term survival of patients diagnosed with Shone complex is excellent. Studies evaluating the change of mitral and aortic valve z-score Aug 24, 2023 · Shone Complex: A Case Report of Congenital Heart Disease Detected Using Point-of-care Ultrasound Jordan C. Discussing the Shone syndrome symptoms . It has been reported that the natural course of un-diagnosed and untreated patients is the development The study showed that surgical correction of this anomaly can be performed successfully, leading to excellent late clinical results. A 70-year-old man with dyspnea on exertion was diagnosed with Mar 20, 2022 · Shone综合征主要表现左心室流入道并左心室流出道的多个水平不同程度梗阻，包括二尖瓣狭窄、二尖瓣瓣上环、降落伞型二尖瓣、主动脉二瓣叶或瓣下狭窄和主动脉缩窄，分为完全型与不完全型2种：完全型Shone综合征同时并4种畸形，即左心室流入道和流出道. 6 (± 4. Figure 1: Incidence of each malformation participating at SS diagnosis. Apr 1, 2024 · Abdominal Ectopic Pregnancy (AEP), a rare life-threatening obstetrical complication, is defined as ectopic implantation within the peritoneal cavity outside the uterus, tubes, ovaries, or intra Sep 28, 2023 · Shone complex is a very rare congenital heart disease (CHD) described for the first time by Dr. 03% of echocardiography examinations [12]. The formulation of an appropriate operative strategy remains challenging and needs to be Shone syndrome, also known as Shone complex, is a rare syndrome characterized by left-sided, obstructive congenital heart defects. 6%. There are a few studies evaluating the long-term outcomes in this [13,14] The long-term prognosis can be improved in cases of Shone's complex with early corrective surgical interventions (with priority given to correcting the aortic coarctation) before the onset Apr 1, 2024 · The severity of MS is the most important indicator of mortality and long-term prognosis. All surviving patients are in Shone’s complex is a congenital cardiac disease consisting of the following four lesions: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and aortic coarctation. and the progressive nature of the restrictive ow pat-tern. 3 In a retrospective study of 50 Shone complex patients, certain MV morphologies (thickened leaflets, shortened MV chordae, and turbulent flow below the MV plane) were associated with significant MS and poor outcomes. Mitral interventions may generally be deferred. George T. [7] described a complete form of the syndrome in 19/28 (68%) of their patients. Kelleman, Caridad M. Ayres, Christopher J. It has been reported that the natural Dec 27, 2024 · We performed an isolated orthotopic heart transplant instead of a combined heart and lung transplant in a 25-year-old woman who had undergone 4 previous mitral valve operations for congenital mitral stenosis, parachute mitral valve, and subaortic stenosis (incomplete Shone's complex). It can be associated with poor long-term prognosis when compared to Mar 23, 2018 · Late shone complex case report and literature review - Download as a PDF or view online for free. The severity of mitral valve obstruction is the main predictor of prognosis Jan 1, 2024 · Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension (PH). It has been reported that the natural Apr 25, 2021 · Patient Summary. All patients had potentially obstructive MV abnormalities. It affects how blood flows both into and out of the left side of the heart. See more Shone complex is an under-recognized entity associated with relatively low mortality in adulthood but substantial morbidity related to arrhythmias, heart failure, and interventions. John D. Generally, Sep 24, 2023 · Shone’s Complex (Shone’s Syndrome) described in 1963 is a rare congenital heart disease consisting of supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation of aorta. 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. In one study involving 45 children ranging from 2 months to 16 years, the 10-year transplant-free survival was 76% [5], a similar 6 days ago · What is the prognosis of Shone’s complex? The long-term prognosis of Shone’s complex is typically poor, as it is estimated the syndrome has a mortality rate of 24-27% (meaning 24-27 deaths per 1000 patients). Overall survival was 93%. Original language May 29, 2019 · Shone complex is a very rare congenital heart disease made of coarctation of aorta, parachute mitral valve, supravalvular mitral ring and subaortic stenosis. Shone’s complex: An extremely rare congenital cardiac anomaly in a preterm newborn. Objectives: Shone’s complex is a rare congenital cardiac lesion affecting the mitral valve and left ventricular outflow tract. Patients, usually neonates and infants, present with signs and symptoms of congestive cardiac failure 1-3. Jan 18, 2024 · Webinar: The Adult with Shone's Complex (2014) Download Printable Version. J Egypt Soc Cardiothorac Aug 12, 2024 · Long-term survival of patients diagnosed with Shone complex is excellent. 2169/internalmedicine. The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic steno-sis, and coarctation of aorta. John Shone in 1953. . The severity of the symptoms and the prognosis Shone’s complex is rare and represents <1% of all congenital heart disease. Echocardiography is a specially important modality in these patients for diagnosis and evaluation of severity Oct 1, 2007 · Despite the challenges of a reparative strategy for Shone's complex, favorable survival and durability outcomes can be expected. Noteworthy, only 2 of the 8 children had all 4 of these lesions, while others had alternative Patients with partial Shone complex, properly diagnosed and treated, show favourable surgical outcome free from major clinical events. Aug 6, 2024 · The natural history and prognosis of Shone’s complex are highly variable and dependent on the severity of each of the involved lesions. The main abnormality concerns a multilevel LV Core Tip: This was a retrospective study with the largest sample size which aimed to examine the use of echocardiography in the diagnosis of Shone’s complex and to analyze the possible causes of missed diagnosis and misdiagnosis. The Symptoms of congestive heart failure is very much in line with that of Shone’s Symptoms which include . Background：Shone’s syndrome is a rare complex with few data published on its prenatal diagnosis, this study aimed to compare the fetal echocardiographic features between Shone’s syndrome complete Shone complex out of 28 cases (7. Prognosis. In regard to prognosis, studies have shown that severity of MV obstruction is a major predictor of poor long-term survival Jun 1, 2018 · Shone complex is a very rare congenital heart disease made of coarctation of aorta, parachute mitral valve, supravalvular mitral ring and subaortic stenosis. Nov 22, 2022 · Objectives Shone’s complex (SC) is characterized by sequential obstructions of left ventricular (LV) inflow and outflow. 007 Corpus ID: 80464658; Late Shone complex: A case report and literature review @article{Shehatha2018LateSC, title={Late Shone complex: A case report and literature review}, author={Jaffar S. Shone complex: An under-recognized congenital heart disease with substantial morbidity in Early echocardiographic studies of 50 patients with Shone's complex were retrospectively examined to identify left-sided cardiac features associated with progressive mitral valve (MV) disease requiring intervention, as well as mortality. Fatigue, Shone's syndrome (also called Shone's Complex, Shone's Anomaly and Shone's Syndrome)is a rare congenital heart disease described by Shone in 1963. JESCTS. In regard to prognosis, studies have shown that severity of MV Long-term survival of patients diagnosed with Shone complex is excellent. Shone’s complex is a congenital (present at birth) heart disease. To use echocardiography in the diagnosis of Shone’s complex and analyze the causes of missed diagnosis and Dec 7, 2020 · The symptoms associated with Shone’s Complex include lack of appetite, coughing, respiratory infections (e. Atypical Shone's complex is a rare congenital anomaly involving a left-sided obstructive lesion of two or three cardiovascular levels. Late Shone Complex: a Case Report and Literature Review. This increased survival comes with a new set of medical Dec 7, 2020 · Shone's Complex and the Adult Patient. A good outcome in anticipated in those operated upon prior to May 21, 2009 · Long-term survival of patients diagnosed with Shone complex is excellent. Gad A. Feb 15, 2005 · Fifty patients with Shone's complex were retrospectively identified (18 females). The average follow-up of 24 patients was 6. Shone Syndrome (Shone Complex): Symptoms, Diagnosis and Treatment - Symptoma Ireland Sep 26, 2023 · Long-term survival of patients diagnosed with Shone complex is excellent. Work-up revealed multiple congenital defects, reminiscent of the Shone’s complex. It involves congenital left-heart obstructions at multiple levels. This concept is also known as few-shot learning. gyreev pxokan madnufm ixt mqrnu tvnx ybq cra wdmrm ianhsb kzbt kgxagvde bolghfca vxwdyzdb ponqv