Agenesia Pericárdica Completa. Presentación de una caso y revisión de la literatura / Complete pericardial agenesis. Presentation of a case and review of the literature

Resumen Paciente masculino de 18 años, quien es valorado en el Servicio de Cardiología del Hospital Víctor Manuel Sanabria Martínez referido del primer nivel de atención por cuadro crónico de dolor torácico atípico acompañado de pectus excavatum y desplazamiento izquierdo del latido de punta a la exploración física. La radiografía de tórax posteroanterior evidencia una cardiomegalia moderada con levocardia máxima. El ecocardiograma transtorácico con función sistólica biventricular conservada, dilatación moderada del ventrículo derecho, sin signos de hipertensión pulmonar e imagen cardiaca de "corazón en lágrima". Angiotomografía computarizada de tórax con contraste que documenta malformación de la caja torácica con hundimiento de la región esternal, en relación con pectus excavatum. Corazón aumentado de tamaño en relación con cardiomegalia grado I y desplazamiento hacia el hemitórax izquierdo. No se observa pericardio en ninguna región. Aurícula derecha levemente dilatada con un ventrículo derecho con diámetro aumentado en su porción media y deformidad de su tracto de salida debido a la forma de la caja torácica, de tal manera que se corrobora el diagnóstico de una agenesia pericárdica completa.

Abstract An 18-year-old male patient who is evaluated in the Cardiology Department of the Víctor Manuel Sanabria Martínez Hospital, referred to the first level of attention due to chronic symptoms of atypical chest pain accompanied by pectus excavatum and left displacement of the peak beat. Chest X-ray Posteroanterior with moderate cardiomegaly with maximum levocardia. Transthoracic echocardiogram with preserved biventricular systolic function, moderate dilatation of the right ventricle, without signs of pulmonary hypertension and cardiac image of "tear heart". Thoracic Computed Angiotomography with contrast documenting malformation of the rib cage with sinking of the external region, in relation to pectus excavatum. Heart enlarged in relation to grade I cardiomegaly and displacement towards the left hemithorax. No pericardium is observed in any region. Slightly dilated right atrium with a right ventricle with an increased diameter in its middle portion and deformity of its outflow tract due to the shape of the rib cage. Corroborating the diagnosis of complete pericardial agenesis.

Clinics in diagnostic imaging (160). Levocardia with abdominal situs inversus

Levocardia (left-sided cardiac apex) with abdominal situs inversus is extremely rare. This is also known as isolated levocardia and is almost always associated with severe forms of congenital heart defects with poor prognosis. We report isolated levocardia in a 13-year-old symptomatic male patient. The purpose of this paper is to outline the imaging features of isolated levocardia and to highlight the role of cardiovascular magnetic resonance imaging (CMR) in the diagnosis and management of such cases. Other forms of cardiac malposition, including dextrocardia, mesocardia and criss-cross heart, with chest radiograph and CMR correlation, are also discussed.

Pancreaticoduodenectomy performed in a patient with situs ambiguous accompanied with isolated levocardia, malrotation, and normal spleen

We report a case of common bile duct (CBD) cancer, successfully managed with pancreaticoduodenectomy, in a patient with isolated levocardia, malrotation, and situs ambiguous (without splenic anomalies). A 59-year-old male patient was referred to Chung-Ang University Hospital with epigastric pain and jaundice. CT and MRI revealed distal CBD cancer without significant lymphadenopathy. Multiple abdominal anatomic anomalies were identified preoperatively, whereas no anatomic anomalies were detected within the chest. The patient had a right-sided stomach and spleen, liver at the midline, several vascular variations around the celiac axis, and intestinal malrotation, but the inferior vena cava and portal vein were normal. A pancreaticoduodenectomy was performed to treat the cancer. The postoperative course was favorable, and the patient was started on combined chemotherapy and radiotherapy 15 days after the surgery. In this case study, we report that pylorus preserving pancreaticoduodenectomy was successful for distal CBD cancer in a patient with rare situs anomalies.

A Case of Unusual Visceral Heterotaxy Syndrome with Isolated Levocardia

Situs inversus of the abdominal organs in the presence of normally placed heart on the left side of the thorax is known as situs inversus with isolated levocardia. This rare condition is commonly associated with severe congenital defects of the heart. We report a case of situs inversus with levocardia in a 19-year-old asymptomatic male patient with completely normal heart on the left chest. Spiral computed tomography of the thorax and abdomen and echocardiographic studies revealed situs inversus of abdominal organs, normal heart (levocardia), mirrored left lungs, a midline liver, a left-sided inferior vena cava connecting to the right atrium, multiple splenic masses in the abdominal right upper quadrant, and aneurysmal dilatation of a splenic artery.

Tectocerebellar dysraphia and occipital encephalocele: an unusual association with abdominal situs inversus and congenital heart disease.

Tectocerebellar dysraphia is a rare constellation of malformations comprising of occipital encephalocele, aplasia of the cerebellar vermis and deformity of the tectum. We describe a 7 month old infant who presented with tectocerebellar dysraphia associated with double outlet right ventricle, pulmonary stenosis and abdominal situs inversus. This association has not been reported in the literature, to the best of our knowledge.

Embryonal biliary atresia with levocardia and situs inversus: a case report

Biliary atresia [BA] is a rare disease and the end result of a destructive inflammatory process in bile ducts, leading to fibrosis and liver cirrhosis. It has two forms: 1] syndromic or fetal or embryonic [10-35%] with various congenital anomalies, 2] non syndromic [70-90%], in which BA is an isolated anomaly. In this article we report on an infant with the first form of BA in which diagnosis and operation was not based on routine liver biopsy but on associated malformations and clinical features that were highly suggestive of embryonal form of biliary atresia. A 70-day old infant with syndromic BA, levocardia, situs inversus and polysplenia. He developed jaundice in 4th day of life, liver was not palpable. Kasai operation was not effective. He developed liver cirrhosis at 3 months of age. Syndromic type of EHBA is a very rare disease with a worse outcome than non syndromic type of BA. Early diagnosis is important and may be difficult as the liver sometimes could not be palpable because of its malposition

Splenic abscess in a boy with isolated levocardia.

Levocardia with situs inversus is a rare condition. We present the case of a fourteen-year-old boy with congenital cyanotic heart disease, isolated levocardia and splenic abscess. It is the first report of both these conditions occurring in the same person.

Duodenal obstruction with isolated levocardia.

We report a patient with isolated levocardia without cardiac or splenic defects associated with duodenal membrane.

Radiodiagnóstico das malposiçöes cardíacas e cardioviscerais: apresentaçäo de 100 casos / Radiological diagnosis of cardiac and cardiovisceral malpositions: report of 100 cases

O "situs" ou posiçäo do coraçäo e de órgäos e vísceras tóraco-abdominais foi determinado com radiografias frontais e oblíquas do tórax e parte superior do abdome em 100 casos, tendo ou näo cardiopatias congênitas ou adquiridas ou pneumopatias. Em alguns casos praticou-se também a tomografia ou a broncografia visando o estudo da morfologia dos brônquios para determinaçäo do "situs" pulmonar e conseqüentemente, atrial. Foi analisada a relaçäo entre os achados radiológicos e o eletrocardiograma em todos os casos. As malposiçöes e anomalias cardiovasculares foram confirmadas por meio do cateterismo cardíaco, cineangiocardiografia e, em alguns casos, durante a cirurgia ou também a necropsia. Pesquisa de corpúsculos de Howell-Jolly foi efetivada em 3 casos com asplenia. Finalmente, consultou-se a literatura sobre outros métodos de diagnóstico com raios x ou ultra-som na detecçäo das malposiçöes do coraçäo e de orgäos e vísceras torácicos e abdominais

Radiological evaluation of double-outlet right ventricle: an analysis of cinecardioangiography in 44 cases

Double-outlet right ventricle is defined as follows: both great arteries arise completely or nearly completelyfrom the right ventricle; neither semilunar valve is in fibrous continuity with either atrioventricular valve; andusually a ventricular septal defect is present and the only outlet from the left ventricle. A total of 44 cases ofdouble-outlet right ventricle is analyzed, in which cineangiocardiographies were done at the Department ofRadiology, Seoul National University Hospital in recent 4 year and 6 months, with specific reference to thesegmental combinations, the height of conus the relationship of great arteries, the location of ventricular septaldefects, and associated anomalies. The resuslts were as follows; 1. Among 44 cases, 36 cases had normal cardiacposition, 4 cases had dextrocardia with situs inversus, 2 cases had dextrocardia with situs solitus, 1 case hadlevocardia with situs inversus, and another 1 case had mesocardia with situs ambiguus. 2. Segmental sets were[S.D.D] in 36 cases, [I.L.L] in 3 cases, [I.D.D] in 2 cases, [S.D.L], [S.L.L] and [A,D,D] in 1 case respectively.3. All cases had bilateral conus. Aortic valve rings were same level as pulmonary valve rings in 25 cases, lowerthan pulmonary valve rings in 17 cases in which 15 cases were type A., and higher than pulmonary valve rings in 2cases. 4. The relation of the great arteries were normal in 15 cases, side-by side in 13 cases, dextromalpositionin 13 cases, and levomal position in 3 cases. 5, The position of the ventricular septal defects with respect tothe origins of the great arteries is subaortic (type A & type B) in 23 cases, subpulmonary (type C) in 13 cases,double committed (type D) in 3 cases, and uncommitted (type E) in 5 cases. 6. Associated cardiac malformations arepulmonary stenosis in 24 which had all cases of type A and type E, aortic stenosis in 6 which were only in type C,left SVC in 6, abnormality of atrioventricular valve in 5, single coronary artery in 4, interrupted IVC in 1,obstructive VSD in 2, ASD in 4, PDA in 4, right aortic arch with levocardia in 5, and ectopic spleen withmesocardia in 1 case. 7. Biplane cinecardioangiogram must be performed in both ventricles to define the VSD andits relationship to the great arteries, and, if necessary, should also be performed in the aorta to rule outcoarctation and coronary artery abnormalities, and in the pulmonary artery to visiualize pulmonary venous returnand mitral valve. Angiography is of crucial importance in differentiating double-outlet right ventricle fromtetralogy of Fallot and complete transposition of the great arteries.

Levocardia isolada com discordancia hepato-atrial, comunicacao interventricular e persistencia do canal arterial.Relato de um caso. / Isolated levocardia with hepatoatrial discordance, interventricular communication and patent ductus arteriosus. Report of a case

Os autores relatam um caso de levocardia isolada com discordancia hepato-atrial, comunicacao interventricular e persistencia do canal arterial, em um paciente com 15 anos de idade, do sexo masculino. A correcao cirurgica foi bem sucedida. Comentam a abordagem diagnostica a esse tipo de anomalia e revisam a literatura

Corrected transposition of the great arteries

The corrected transposition of the great arteries is an usual congenital cardiac malformation, which consisitsof transposition of great arteries and ventricular inversion, and which is caused by abnormal developement ofconotruncus and ventricular looping. High frequency of associated cardiac malformations makes it difficult to getaccurate morphologic diagnosis. A total of 18 cases of corrected transposition of the great arteries is presented,in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul NationalUniversity Hospital between Sep. 1976 and June 1981. The clinical, radiogrpahic ,and operative findings with theemphasis on the angiocardiographic findings were analized. The resuls are as follows; 1. Among 18 cases, 13 caseshave normal cardiac position, 2 cases have dextrocardia with situs solitus, 2 cases have dextrocardia with situsinversus and 1 case has levocardia with situs inversus. 2. Segmental sets are {S.L.L.} in 15 cases, and {I.D.D} in3 cases and there is no exception to loop rule. 3. Side by side interrelationships of both ventricles and bothsemilunar valves are noticed in 10 and 12 cases respectively. 4. Subaortic type conus is noted in all 18 cases. 5.Associated cardiac malformations are VSD in 14 cases, PS in 11, PDA in 3, PFO in 3, ASD in 2, right aortic arch in2, tricuspid insufficiency, mitral prolapse, persistent left SVC and persistent right SVC in 1 case respectively.6. For accurate diagnosis of corrected TGA, selective biventriculography using biplane cineradiography is anessential procedure.