RESUMEN
Situs inversus incompletus is a rare congenital condition in which the major abdominal organs are reversed or mirrored from their normal positions. It is often associated with multiple congenital anomalies. We present the case of a 38-year-old woman with dyspnea and a clinical history of chronic kidney disease and kidney transplantation. Echocardiography showed a right atrial mass, and analysis of multidetector computed tomography angiography revealed the interruption of the inferior vena cava with an increase of the azygos vein and azygos continuation. These congenital malformations are often associated with deep vein thrombosis and/or pulmonary thromboembolism and explained the occurrence of dyspnea. Cardiac magnetic resonance with contrast medium confirmed the presence of the right atrial mass, the characteristics of which were attributed to interatrial thrombus, which was further confirmed by the success of thrombolytic therapy and the remission of symptoms. In conclusion, we described a case of situs inversus with levocardia in association with infrahepatic interruption of the inferior vena cava, and azygos continuation with cardiac thrombus and chronic renal failure.
Asunto(s)
Vena Ácigos/anatomía & histología , Levocardia/complicaciones , Trombosis/etiología , Vena Cava Inferior/anomalías , Adulto , Femenino , HumanosRESUMEN
Aneurysm of the sinus of Valsalva, a rare cardiac condition, results from dilation of an aortic sinus. Sudden aneurysm rupture can trigger rapidly progressive heart failure. We discuss the case of a 57-year-old woman with situs ambiguus, isolated levocardia, and polysplenia who presented with acute-onset heart failure. Transesophageal echocardiograms revealed an aneurysm of the right coronary sinus of Valsalva that had ruptured into the right atrial cavity. The patient underwent successful surgical repair. To our knowledge, this is the first report of a sinus of Valsalva aneurysm in a patient with this combination of congenital abnormalities. We briefly review the association between congenital heart disease, situs ambiguus, and ciliary dysfunction.
Asunto(s)
Anomalías Múltiples , Rotura de la Aorta/etiología , Síndrome de Heterotaxia/complicaciones , Levocardia/complicaciones , Seno Aórtico , Bazo/anomalías , Enfermedades del Bazo/complicaciones , Rotura de la Aorta/diagnóstico , Ecocardiografía Transesofágica , Femenino , Humanos , Levocardia/diagnóstico , Persona de Mediana Edad , Bazo/diagnóstico por imagen , Enfermedades del Bazo/congénito , Enfermedades del Bazo/diagnóstico , Tomografía Computarizada por Rayos XRESUMEN
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.
Asunto(s)
Absceso Abdominal/diagnóstico , Cianosis/diagnóstico , Levocardia/diagnóstico , Situs Inversus/diagnóstico , Enfermedades del Bazo/diagnóstico , Absceso Abdominal/complicaciones , Adolescente , Comorbilidad , Cianosis/complicaciones , Cardiopatías Congénitas , Humanos , Levocardia/complicaciones , Masculino , Situs Inversus/complicaciones , Enfermedades del Bazo/complicacionesRESUMEN
A 69-year-old man with situs inversus, levocardia and inverted great arteries developed severe dynamic left ventricular outflow tract (LVOT) obstruction with systolic anterior motion of the anterior mitral leaflet. There was no asymmetric septal hypertrophy. A possible mechanism of the LOVT obstruction in the present case may have been related to an abnormally long and bent outflow tract resulting from overriding of the right ventricle over the LVOT due to a congenital malposition of the heart. Mitral valve replacement with septal myectomy was performed in order to eliminate systolic anterior motion. The postoperative course has been excellent.
Asunto(s)
Levocardia/complicaciones , Levocardia/diagnóstico por imagen , Obstrucción del Flujo Ventricular Externo/complicaciones , Obstrucción del Flujo Ventricular Externo/diagnóstico por imagen , Anciano , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Humanos , Levocardia/cirugía , Masculino , Tomografía Computarizada por Rayos X , Obstrucción del Flujo Ventricular Externo/cirugíaRESUMEN
Six children, aged 12 days to 13 years, with left isomerism and complete atrioventricular (A-V) block are presented. In all six patients the diagnosis of left isomerism was suggested by an interrupted inferior vena cava found during cardiac catheterization and angiocardiography; four patients had complex heart disease consisting of endocardial cushion defect, five had a common atrium, three had pulmonary stenosis, three had patient ductus arteriosus and two had dextrocardia. Further anatomic abnormalities included situs inversus of the viscera (four patients) as well as partial malrotation of the bowel. Of the six patients, four had congenital complete A-V block, whereas the remaining two had A-V conduction disturbances documented during early infancy that progressed to complete A-V block later in life. All six patients required pacemaker implantation and five of the six patients died. This report discusses the clinical presentation of complete AV block and left isomerism and reviews the literature.
Asunto(s)
Bloqueo Cardíaco/complicaciones , Bazo/anomalías , Adolescente , Arritmias Cardíacas/terapia , Estimulación Cardíaca Artificial , Niño , Preescolar , Dextrocardia/complicaciones , Electrocardiografía , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/patología , Humanos , Lactante , Recién Nacido , Levocardia/complicaciones , Radiografía , Síndrome , Venas Cavas/anomalíasRESUMEN
Two cases of complex congenital heart disease are described in which systemic and pulmonary bloodstreams crossed at the atrioventricular level. Both patients were examined clinically, echocardiographically, and by cardiac catheterization, including angiography, and both underwent cardiac surgery and had intraoperative mapping of their conduction systems. Both patients were found to have levocardia and situs solitus of viscera and atria, large ventricular septal defect, and straddling right atrioventricular valve. One patient had atrioventricular discordance but with the left ventricle anterior and alightly on the right and with pulmonary atresia and dextromalposition of the aorta. The other patient had atrioventricular concordance but with the left ventricle inferior and slightly on the left and with ventricular-arterial concordance to normally related great arteries and banded pulmonary trunk. To our knowledge, this combination including straddling right atrioventricular valve has not been reported in the literature before. In both patients the straddling atrioventricular valve was thought to preclude corrective operation at that time. A unique palliative procedure--left ventricle-to-pulmonary trunk shunt--was successful in the first case and partial debanding of the pulmonary trunk in the second.
Asunto(s)
Cardiopatías Congénitas/complicaciones , Defectos del Tabique Interventricular/complicaciones , Levocardia/complicaciones , Válvula Mitral/anomalías , Válvula Tricúspide/anomalías , Cateterismo Cardíaco , Niño , Preescolar , Ecocardiografía , Electrocardiografía , Femenino , Defectos del Tabique Interventricular/diagnóstico , Humanos , Levocardia/diagnósticoRESUMEN
A simple intraatrial reconstruction can be utilized in infants to simultaneously correct both the systemic and pulmonary venous returns. Two infants with polysplenia syndrome treated in the first months of life are presented. One had levocardia and combined total anomalous pulmonary venous connection (type II-Cardiac) and anomalous systemic venous connection. The other had dextrocardia L-loop, L-normal great arteries, and combined total anomalous pulmonary venous connection (type II-Cardiac) and anomalous systemic venous connection. The posterior position of the systemic atrioventricular valve and systemic ventricle in both patients allowed the use of this reconstructive technique. The extensive size and transverse position of this patch when used in the infant may require both continued postoperative controlled ventilation until it stiffens and prolonged attention to postoperative cardiac care until adaptation of the infant heart to the new atria can occur. Early restudy and reoperation should be performed if signs of stenosis develop.
Asunto(s)
Dextrocardia/cirugía , Cardiopatías Congénitas/cirugía , Levocardia/cirugía , Venas Pulmonares/cirugía , Dextrocardia/complicaciones , Femenino , Venas Hepáticas/anomalías , Humanos , Recién Nacido , Levocardia/complicaciones , Masculino , Métodos , Venas Pulmonares/anomalías , Venas Cavas/anomalíasRESUMEN
Isolated ventricular inversion with left atrial isomerism, partial anomalous pulmonary venous connection, and interruption of the inferior vena cava with azygos continuation to a right superior vena cava was diagnosed by echocardiography in a neonate. At 48 days of age, the patient underwent successful anatomic correction with redirection of flow from the superior vena cava and hepatic veins to the left-sided tricuspid valve, and flow from the pulmonary veins to the right-sided mitral valve. In the present report, the surgical techniques of this case are described, along with a survey of the surgical literature covering anatomic repair of isolated ventricular inversion.
Asunto(s)
Atrios Cardíacos/cirugía , Ventrículos Cardíacos/anomalías , Levocardia/cirugía , Venas Pulmonares/anomalías , Venas Cavas/anomalías , Ecocardiografía , Femenino , Humanos , Recién Nacido , Levocardia/complicaciones , Levocardia/diagnóstico por imagen , Resultado del TratamientoRESUMEN
BACKGROUND: It is well recognized that a right ventricle (RV) may eventually fail if working as systemic. This has led to arterial switch operation for transposition of the great arteries (TGA), but more recently to anatomic correction of 'corrected transposition' or atrio-ventricular discordance (AVD) combining an atrial switch and a ventricular outflow repair (arterial switch or Rastelli type). METHODS: We report here 8 cases of such anatomic correction of AVD in patients from 3 months to 10 years. Of these patients, 6 had situs solitus, and 2 situs inversus; 7 patients had a ventricular septal defect (VSD); 5 had an increased pulmonary flow, and 3 had a decreased pulmonary flow with pulmonary stenosis (PS) or pulmonary atresia (PA). In all, 6 had discordant and 1 had concordant ventriculo-arterial (VA) relations, and 1 had double-outlet RV, 2 had an associated coarctation, and 1 had a cor triatriatum. Of the 8 patients, 5 had pulmonary artery hypertension, 3 a moderate to severe tricuspid insufficiency and 6 had undergone one or more previous operations. Correction was done between 3 months and 10 years of age, with 7 having a Senning procedure (twice left-sided) and 1 a Mustard type operation. VA outflows were repaired with: 4 arterial switches, 1 left ventricle to aorta rerouting, 1 right ventricular outflow tract (RVOT) repair and 2 Rastelli. A tricuspid annuloplasty was done once. RESULTS: All patients survived the operation, but 2 patients needed a reoperation (both successful): late obstruction of pulmonary venous channel after a left-sided Senning, and residual VSD closure. Mean follow-up is 45 months (3-122). Of the 8 patients, 6 patients are in sinus rhythm, 2 have junctional rhythm. A patient with junctional rhythm and occasional tachycardia died suddenly 18 months after surgery 1 week after satisfactory evaluation. All have a normal left ventricle function as evidenced by echocardiography. CONCLUSIONS: Despite a more demanding procedure, the 'anatomic repair' of hearts with AVD is possible, even in infancy, with good early and mid-term result. Despite the potential late rhythmic problems of the Senning operation (somehow more frequent with AVD in our experience), it may become, with increasing experience, the procedure of choice in corrected transposition.
Asunto(s)
Dextrocardia/cirugía , Levocardia/cirugía , Transposición de los Grandes Vasos/cirugía , Angiografía Coronaria , Dextrocardia/complicaciones , Dextrocardia/diagnóstico por imagen , Defectos del Tabique Interventricular/complicaciones , Defectos del Tabique Interventricular/diagnóstico por imagen , Humanos , Lactante , Levocardia/complicaciones , Levocardia/diagnóstico por imagen , Complicaciones Posoperatorias , Reoperación , Transposición de los Grandes Vasos/complicacionesRESUMEN
Criss-cross heart is an angiographically confusing malformation where apparent crossing but not mixing of contrast medium is seen at atrioventricular level. The malformation can be understood by a clockwise or counter-clockwise ventricular rotation. In the normal heart or one with complete transposition ventricular rotation is clockwise. With corrected transposition rotation occurs in a counter-clockwise fashion. One case of criss-cross heart with associated corrected transposition and two criss-cross hearts occurring with complete transposition are reported.
Asunto(s)
Cardiopatías Congénitas/patología , Levocardia/patología , Transposición de los Grandes Vasos/patología , Corazón/diagnóstico por imagen , Hemodinámica , Humanos , Lactante , Recién Nacido , Levocardia/complicaciones , Levocardia/diagnóstico por imagen , Radiografía , Rotación , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/diagnóstico por imagenRESUMEN
An unusual case of isolated levocardia, duodenal obstruction, and malrotation with volvulus is reported. Three cases of situs inversus abdominus with malrotation with volvulus have been reported to date. The current case is the fourth case and only the second survivor in the series. In cases of situs inversus abdominus, because of inversion of viscera, the volvulus may occur in an anticlockwise direction and, thus, should be derotated in a clockwise direction.
Asunto(s)
Obstrucción Intestinal/complicaciones , Levocardia/complicaciones , Humanos , Lactante , MasculinoRESUMEN
An unusual case of duodenal obstruction in an infant with isolated levocardia is described. The following anatomic features were shown preoperatively: (1) duodenal obstruction; (2) right-sided stomach and spleen; (3) left-sided liver; (4) totally right-sided located colon in mirror image nonrotation; and (5) levocardia without any defect or dysfunction. The mentioned findings were verified intraoperatively and the cause of the duodenal obstruction was found to be an aberrant vessel. This case is a rare one regarding the lack of heart and spleen defects and the cause of duodenal obstruction. It also demonstrates the necessity of meticulous investigation and precise description of the anomaly and suggests individualization as far as the management of these cases is concerned.
Asunto(s)
Obstrucción Duodenal/congénito , Levocardia/complicaciones , Situs Inversus/patología , Obstrucción Duodenal/complicaciones , Humanos , Recién Nacido , MasculinoRESUMEN
Isolated levocardia with situs inversus, or ILSI, is a rare anomaly with a reported incidence of 1:22,000 in the general population and from 0.4% to 1.2% of all patients with congenital heart diseases. It is commonly associated with both congenital heart disease and splenic abnormalities, either asplenia or polysplenia. The prognosis is poor, and only about 5% to 13% of patients survive more than 5 years. The case described here, which had computed tomographic findings, is the first case reported with multiple malignancies associated with ILSI. This patient, at 73 years of age, is the second longest survivor reported in the literature.
Asunto(s)
Anomalías Múltiples , Levocardia/complicaciones , Neoplasias Primarias Múltiples/complicaciones , Situs Inversus/complicaciones , Anomalías Múltiples/diagnóstico , Anomalías Múltiples/mortalidad , Anciano , Femenino , Humanos , Levocardia/diagnóstico , Levocardia/mortalidad , Neoplasias Primarias Múltiples/mortalidad , Pronóstico , Situs Inversus/diagnóstico , Situs Inversus/mortalidad , Tasa de Supervivencia , Tomografía Computarizada por Rayos XRESUMEN
We report a patient with isolated levocardia without cardiac or splenic defects associated with duodenal membrane.
Asunto(s)
Enfermedades Duodenales/complicaciones , Obstrucción Intestinal/complicaciones , Levocardia/complicaciones , Humanos , Recién NacidoRESUMEN
Authors report a case of preduodenal portal vein in a woman 68 years old with acute cholecystitis in situs viscerum inversus adbominis and levocardia. Surgery was difficult. They shortly analyze also etiology and clinical manifestations of situs inversus and PPV, with related malformations. They conclude that it is important to recognize the presence of situs inversus preoperatively and everytime suspect other anatomical anomalies.
Asunto(s)
Anomalías Múltiples , Colelitiasis/complicaciones , Cardiopatías Congénitas/complicaciones , Levocardia/complicaciones , Vena Porta/anomalías , Situs Inversus/complicaciones , Anciano , Colecistectomía , Colecistitis/etiología , Colecistitis/cirugía , Colelitiasis/cirugía , Duodeno/anomalías , Femenino , HumanosRESUMEN
Isolated levocardia (IL) is a rare type of situs inversus in which the heart is in the normal left-side position, but the abdominal viscera are in the dextroposition. Polysplenia is a congenital disorder affecting the asymmetric organs, including the heart, lungs, bronchi, liver, stomach, intestines, and spleen. In this report, we present a rare type satisfying the characteristics of both IL and polysplenia, confirmed by several imaging investigations, which revealed normally structured lungs and heart and the existence of the inferior vena cava (IVC), which runs on the left side, returns to the anterior-right side at the liver, and returns to the right atrium directly. Anatomical investigation and careful observation will make it possible to improve the prognosis of IL.