shones complex syndrome

A parachute mitral valve (PMV) is a congenital mitral valve anomaly diagnosed in infancy, and it can also be discovered in adults during echocardiography. Surgical management is common in infants to prevent complications from left-heart obstructions. In adults, PMV may be found independently or with other cardiac defects. Prophylactic antibiotics are recommended for certain congenital heart anomalies before dental procedures. A study suggests reconsidering guidelines to include anomalies like bicuspid aortic valve and MVP for antibiotic prophylaxis...

BACKGROUND: The decision to perform biventricular repair (BVR) in neonates and infants presenting with either single or multiple left ventricle outflow obstructions (LVOTOs) and a borderline left ventricle (BLV) is subject to extensive discussion, and limited information is known regarding the long-term outcomes. As a result, the objective of this study is to critically assess and summarize the available data regarding the prognosis of neonates and infants with LVOTO and BLV who underwent BVR...

Complex congenital heart disease (CHD) in each of dichorionic diamniotic (DiDi) twin pairs is extremely rare and has not been well characterized. Four DiDi twin pairs were included in this multi-institutional case series. The congenital cardiac abnormalities noted included tetralogy of Fallot (ToF) with pulmonary atresia and collaterals (n = 1), ToF with absent pulmonary valve (n = 1), ToF (n = 2), discontinuous right pulmonary artery (RPA) (n = 1), tricuspid atresia (TA) with normally related great arteries and pulmonary valve stenosis or atresia (n = 2) and coarctation of aorta (CoA) with bicuspid aortic valve (BAV) and borderline left-sided structures (n = 1)...

Shone syndrome is a rare and complex congenital heart disease. It is characterized in its complete form, by a constellation of the four potentially obstructive lesions of the left heart side. Incomplete Shone's complex includes 2 or 3 of the characteristic lesions which, include a supravalvular mitral membrane, valvular mitral stenosis (MS) by a parachute mitral valve (PMV), subaortic stenosis and aortic coarctation (Coa). We describe here, the case of a 35-year-old man, hospitalized for chronic dyspnea and in whom, the diagnosis of incomplete Shone's complex is retained by echocardiographic analysis...

We aim to share the diagnostic methods and relevant findings of a rare case involving a 1-year-old girl with a variation of Shone syndrome, presenting with the coexistence of a single coronary artery and a fistula between the coronary artery and the right pulmonary artery. The patient had previously undergone aortic arch reconstruction surgery and during the preoperative evaluation, cardiac computed tomography angiography (CCTA) revealed the additional presence of a single coronary artery originating from the right, which fistulized into the right pulmonary artery distally...

BACKGROUND: While early outcomes of the Konno-Rastan and modified Konno procedures are known, long-term outcomes remain undetermined. Our objective was to examine long-term clinical and echocardiographic outcomes. METHODS: Retrospective review was performed of patients undergoing Konno-Rastan and modified Konno for complex left ventricular outflow tract (LVOT) obstruction from 1/1980-1/2021. The Ross-Konno procedure was excluded due to its often limited ventriculotomy...

KEY CLINICAL MESSAGE: This case underlined the importance of having high suspicion for lobar hemorrhage as a rare but deadly complication of herpes simplex virus encephalitis and shone light upon the added complexity it poses on management on an already deadly disease. ABSTRACT: Herpes simplex virus (HSV) encephalitis is the most common type of sporadic encephalitis that inflicts high rates of morbidity and mortality. Differentiating a progressing encephalitis syndrome from a lobar hemorrhage as a complication presents a challenge and requires great vigilance and insight on part of the treating physician...

Congenital heart disease (CHD) remains a predisposing cardiac condition for infective endocarditis (IE). Case report: We present the case of 8-year-old boy with no known pre-existing cardiac disease diagnosed with infective endocarditis (IE) with Gemella sanguinis. After admission, he underwent transthoracic echocardiography (TTE), which revealed the presence of Shone syndrome with a bicuspid valve, mitral parachute valve and severe aortic coarctation. He developed a paravalvular aortic abscess with severe aortic regurgitation and left ventricle (LV) systolic dysfunction for which he required a complex surgical intervention after six weeks of antibiotic treatment, consisting of Ross operation and coarctectomy, with a complicated postoperative course, cardiac arrest and ECMO support for five days...

This is the first description of active clinical manifestation of endocardial fibroelastosis (EFE) and remodeling of the endocardium via endothelial-to-mesenchymal transformation (EndMT) in an adolescent with Shone's variant hypoplastic left heart complex (HLHC) and a genetic heterozygous ABL1 variant. While EFE has not been typically associated HLHC or Shone's syndrome, in this patient flow alterations in the left ventricle (LV), combined with genetic alterations of intrinsic EndMT pathways led to active clinical manifestation of EFE in adolescence...

BACKGROUND: Shone's syndrome is a rare complex congenital anomaly. The classical definition consists of four anomalies: supravalvular mitral membrane, parachute mitral valve (PMV), subaortic stenosis, and coarctation of the aorta (CoA). Few studies have been reported on Shone's syndrome in adults, particularly the primary surgical correction of the anomalies. CASE PRESENTATION: A 22-year-old female patient presented with chest distress and tachypnea. Echocardiography and CT revealed supravalvular mitral membrane, PMV, Bicuspid aortic valve stenosis, CoA and patent ductus arteriosus...

BACKGROUND: Shone's complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients. AIM: To use echocardiography in the diagnosis of Shone's complex and analyze the causes of missed diagnosis and misdiagnosis. METHODS: This was a retrospective study of patients who underwent echocardiography and repair surgery from February 14, 2008, to November 22, 2019. The patients were followed once a year at the outpatient clinic after surgery...

A female infant, who was diagnosed antenatally with complex heart disease, confirmed to be Shone's complex postnatally, underwent bilateral pulmonary artery banding, patent ductus arteriosus stent insertion and balloon aortic valvuloplasty soon after birth. She was found to have bilateral megaureters, left hydronephrosis and asplenia. She was on lifelong prophylactic antibiotics and extra vaccines. She had two episodes of pseudo-obstruction of the small bowel, but barium follow-through was normal. She also had a large bowel obstruction and work-up for Hirschsprung disease confirmed the diagnosis...

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 valve, and coarctation of the aorta (CoA), which affects only a small minority of people. CASE PRESENTATION: We report the case of a 25-year-old woman with a past medical history of moderate mitral stenosis, since she was 10-year-old with uncontrolled high blood pressure, treated with nicardipine...

Parachute mitral valve (PMV) is a rare congenital cardiac valvular anomaly often associated with other congenital cardiac defects, particularly Shone's complex, but may infrequently occur in isolation. PMV and its variants are predominantly associated with mitral stenosis (MS) or rarely mitral regurgitation (MR). We present the case of a middle-aged female who was evaluated for a syncopal episode and found to have an atypical variant PMV with disproportionately long anterior mitral leaflet, bileaflet mitral valve prolapse, and mitral annular disjunction, without associated MS or MR...

BACKGROUND: Aortic coarctation (AoCoa) is a congenital stenosis of aorta, which often co-exists with other congenital heart defects. Many studies have shown the importance of regular follow-up in these patients however there is scarcity of knowledge relating to the impact of left ventricle inflow lesions (LVIT) and left ventricle outflow track lesions (LVOT). The aim of this study is to evaluate the impact of isolated AoCoa with LVIT and/or LVOT on haemodynamic, morbidity and mortality...

Objective: Infants requiring mitral valve replacement have few viable options. Recently, stented bovine jugular vein graft (Melody) has been surgically implanted in such cases. Herein, we report our experience, elaborating on evolution of implantation technique, pitfalls, as well as long-term outcome (including late dilatability). Methods: Seven Melody valves were implanted (2013-2019). The median patient age and weight were 6.7 (1.8-30.5) months and 5.8 (4.6-9...

BACKGROUND: Complex cardiac anomalies are a set of associated heart structural anomalies requiring some adaptive mechanism to maintain life. OBJECTIVES: This is to determine pattern and prevalence of common complex cardiac anomalies among children presenting for cardiac surgery in Enugu. METHODOLOGY: The children that present for cardiac surgery in our center over a six-month period were admitted and rescreened using a standardized echocardiographic method, information on biodata, oxygen saturation and 2D echo diagnosis were recorded in a proforma...

A 22-year-old male had a dual chamber permanent pacemaker (PPM) implanted for complete heart block following aortic valve replacement for Shone's syndrome 3 months previously. He presented with acute shortness of breath due to severe para-valvular leak with aortic valve dehiscence following a motor vehicle accident. He was scheduled for redo sternotomy and a pre-operative PPM check was undertaken. A programmer wand (Model 2067, Medtronic Inc., Minneapolis, MN, USA) was applied to the patient's PPM site to perform interrogation...

BACKGROUND: Shone's syndrome is a rare complex congenital cardiac condition, characterized by a supra-valvular mitral ring, parachute deformity of the mitral valve, aortic stenosis, and coarctation of the aorta. CASE: A 26-year-old parturient with partial Shone's syndrome presented to our delivery unit in pulmonary edema. She underwent a scheduled cesarean section performed under a combined spinal-epidural anesthetic at 33 weeks. She had multidisciplinary input from the cardiac, obstetric, and anesthetic teams, which led to a good outcome...

Kleefstra syndrome (chromosome 9q34.3 deletion) is a rare genetic disorder with less than 110 patients reported till date. We report a 4-month-old Caucasian girl with Kleefstra syndrome and Shone's complex, an association which has not been previously reported. Surgical planning for patients with Kleefstra syndrome and complex CHD can pose challenges due to an uncertain natural history and a risk of post-operative pulmonary hypertension.