ABSTRACT
Abstract Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.
Subject(s)
Humans , Female , Middle Aged , Endocardial Cushion Defects/surgery , Heart Septal Defects/surgery , Mitral Valve/abnormalities , Treatment Outcome , Heart Valve Prosthesis Implantation/methods , Endocardial Cushion Defects/complications , Mitral Valve Annuloplasty/methods , Heart Septal Defects/complications , Mitral Valve/surgeryABSTRACT
El síndrome de Ellis-Van Creveld (SEVC) o displasia condroectodérmica se debe a una mutación de transmisión autosómica recesiva en el brazo corto del cromosoma 4 y afecta múltiples órganos. Descrito como una tétrada clásica de condrodisplasia, displasia ectodérmica, polidactilia y defectos cardíacos congénitos, sólo se conoce a partir de informes y series de casos. Se describe el caso de un varón de 3 meses, sin antecedentes familiares de importancia, que presentó un cuadro de condrodisplasia, labio superior fusionado al paladar, simpolidactilia posaxial bilateral en las manos, displasia del desarrollo de las caderas, tórax estrecho con costillas cortas y compromiso cardíaco. El presente caso sería la primera comunicación del SEVC en el Perú.
Ellis-Van Creveld Syndrome or chondrectodermal dysplasia is produced by an autosomal recessive inheritance secondary to mutation in the short arm of chromosome 4. The syndrome affects multiple organs. It is described as a clinical tetrad that involves chondrodysplasia, ectodermal dysplasia, polydactyly and congenital heart defects. It is only known from reports and case series. We present a three months old male, without relevant family history, who presented chondrodysplasia, upper lip merged to palate, bilateral sinpolydactyly in the hands, developmental dysplasia of the hip, narrow chest with short ribs, and heart defects. This case is the first report of EVC in Peruvian literature.
Subject(s)
Humans , Infant , Male , Abnormalities, Multiple , Ellis-Van Creveld Syndrome/complications , Endocardial Cushion Defects/complications , Heart Atria/abnormalities , Tricuspid Atresia/complicationsABSTRACT
Fístulas entre a aorta e o átrio direito podem ser de origem congênita ou adquirida. As fístulas aortocamerais congênitas são raras anomalias do coração que podem ser confundidas com outros defeitos como comunicações interventriculares ou interatriais. São raros canais vasculares extra cardíacos e sua história natural, apresentação clínica e tratamento adequado ainda são obscuros. As fístulas adquiridas são, em geral, decorrentes de ruptura do seio de valsalva pós-infecciosa. Neste relato apresentamos um caso de difícil diagnóstico ecocardiográfico, no qual o diagnóstico de fístula aorta-átrio direito foi confundido com o de comunicação interventricular perimenbranosa e só confirmado após estudo hemodinâmico.
Aorta to right atrium fistula can be a congenital or condition. Congenital fistulas are uncommon heart diseases and can be mistaken by other heart lesions like interventricular septal defect or atrial septal defect. They seem to be are extra-cardiac vascular channels and their natural history, clinical presentation and treatment remain uncertain. Acquired fistula between aorta and right atrium is usually due to a post infeccious rupture of sinus of valsalva. The authors present a case of aorta-right atrium fistula that had been mistaken by perimenbranous interventricular septal defect during echocardiography examination and confirmed by cardiac catheterization.
Subject(s)
Humans , Female , Adult , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnosis , Endocardial Cushion Defects/complications , Echocardiography/methods , Echocardiography , Fistula/complicationsABSTRACT
A 56-year-old man was evaluated for exertional dyspnoea. Chest X-ray showed mild cardiomegaly and a dilated main pulmonary artery. On echocardiogram he was found to have ostium primum atrial septal defect with moderate tricuspid insufficiency. Cardiac catheterization revealed an oximetry step-up of 14% at low right atrium with angiogram demonstrating a cleft in the mitral valve, an elongated left ventricular outflow tract and ventricular septal defect closed by a septal aneurysm. Coronary angiogram revealed ostial compression of the left main coronary artery with the rest of the coronary artery anatomy being normal.
Subject(s)
Coronary Angiography , Coronary Disease/etiology , Diagnosis, Differential , Echocardiography , Endocardial Cushion Defects/complications , Cardiac Catheterization , Humans , Male , Middle Aged , Pulmonary Artery/pathology , Tricuspid Valve Insufficiency/complications , Ventricular Outflow Obstruction/complicationsABSTRACT
A case of successful surgical repair of tetralogy of Fallot with complete atrioventricular canal in 4 year old female child is reported. Double patch technique and combined right atrial and right ventricular (RV) approach were used to repair this defect. The ventricular patch was tailored wide and redundant anteriorly in the form of a sail to prevent subaortic obstruction. Right ventricular outflow tract obstruction was relieved through an infundibular incision with subsequent use of subannular pericardial patch. The relevant literature on the management of this rare anomaly is briefly reviewed.
Subject(s)
Child, Preschool , Endocardial Cushion Defects/complications , Female , Heart Ventricles/surgery , Humans , Polytetrafluoroethylene , Prostheses and Implants , Tetralogy of Fallot/complicationsABSTRACT
Se presenta el caso de un paciente de 44 años, con un síndrome anginoso, en quien el estudio demostró serología positiva para lúes y la cinecoronariografía una acentuada estenosis de ambos óstiums coronarios. El paciente fallece al día siguiente del examen. Se presentan los resultados del estudio necrópsico. Se comenta la escasa frecuencia actual de las cardiopatías luéticas y las limitaciones de la penicilina en el tratamiento de la sífilis tardía. Se enfatiza que los síndromes coronarios por estenosis de los óstium constituyen indicación de revascularización de urgencia ya que su mortalidad es semejante a la de las lesiones de tronco
Subject(s)
Adult , Humans , Endocardial Cushion Defects/complications , Syphilis, Cardiovascular/complicationsABSTRACT
Durante operaçäo para correçäo de persistência do canal atrioventricular comum parcial, foi detectado na cúspide anterior da valva mitral, nódulo com cerca de 1,5 cm de diâmetro o qual levou à suspeita de aneurisma da valva mitral, diagnóstico confirmado pelo exame anátomo-patológico. Näo havia história de endocardite infecciosa nem sinais de acometimento inflamatório agudo ou crônico do aneurisma. Säo discutidos o diagnóstico, as alteraçöes anátomo-patológicas e apresentados os dados da correçäo cirúrgica