Dextrocardia y herniación intratorácica del hígado. Una rara entidad / Dextrocardia and Intrathoracic Herniation of the Liver. A Rare Entity

Introducción: la incidencia de dextrocardia como anomalía congénita es menor del 0.01% y la combinación con herniación intratorácica del hígado semejando una neoplasia benigna sin antecedente de trauma toracoabdominal abierto o contuso lo hace aún menos frecuente. Caso clínico: se presenta el caso de paciente femenina de 34 años de edad que consulta por dolor de espalda. Al examen físico, se auscultan ruidos cardíacos en el hemitórax derecho y la radiografía de tórax evidencia dextrocardia e imagen que semeja masa supra diafragmática derecha, la TAC trifásica confirma la presencia de protrusión de un segmento del hígado de forma redondeada a través de un defecto no abierto del hemidiafragma derecho. Su tratamiento ha sido conservador. Conclusión: la combinación de dextrocardia acompañada de herniación de una porción del hígado a través de un defecto del diafragma derecho es una asociación extremadamente rara y los reportes de caso publicados son escasos

Human Laterality Disorders: Pathogenesis, Clinical Manifestations, Diagnosis, and Management.

Human laterality disorders comprise a group of diseases characterized by abnormal location (situs) and orientation of thoraco-abdominal organs and vessels across the left-right axis. Situs inversus totalis is mirror image reversal of thoraco-abdominal organs/great vessels. Situs ambiguus, better known as heterotaxy, is abnormal arrangement of thoraco-abdominal organs across the left-right axis excluding situs inversus totalis. Heterotaxy, also referred to as atrial or atrial appendage isomerism, is characterized by abnormal location of left-sided or right-sided organs with loss of asymmetry of normally paired asymmetric organs. It is associated with a variety of anomalies involving the heart, great vessels, lungs and intra-abdominal organs. Right and left atrial isomerism are associated with multiple complex congenital cardiac and vascular anomalies, many of which are lethal when untreated. Isomerism may also affect the lungs, spleen, liver, gall bladder, and intestines. Innovative surgical therapy of heterotaxy/isomerism has reduced early mortality and markedly improved long-term prognosis.

Atrioventricular Canal and Dextrocardia: A Case Report with Implications for Community-Based Providers.

Congenital heart defects (CHD) are the most common congenital malformation reported in the literature, with a global incidence of eight per every 1,000 live births. In the United States approximately 40,000 infants are born each year with a CHD. Of the infants diagnosed with a CHD, one in every four heart defects are life threatening in origin. Early identification and treatment of congenital heart lesions, beginning with a comprehensive physical assessment after birth, are critical. For infants delivered at community-based hospitals, the importance of the physical assessment, timing of diagnostic strategies, anticipatory planning, and interprofessional collaboration among referring and accepting centers cannot be understated. This article presents a rare case of an infant with atrioventricular canal complicated by dextrocardia. Embryology, pathophysiology, epidemiology, symptomology, cardiac assessment, diagnostics, treatment, and nursing strategies for facilitating transfer of care from community-based hospitals to tertiary medical centers are discussed.

Abnormalities of Fetal Situs: An Overview and Literature Review.

The incidence of congenital heart disease is most commonly reported in 8 per 1000 live births. Situs anomalies are among the least common forms of congenital heart disease. This study defines situs and describes the variations of fetal situs anomalies. Situs refers to the arrangement of the viscera, atria, and vessels within the body. Situs solitus describes the normal arrangement; situs inversus describes inverted arrangement of the viscera and atria; and situs ambiguous (also referred to as heterotaxy) describes disturbances in arrangements that can neither be identified as solitus nor inversus. This review will concentrate on heterotaxy, as it is the most complicated situs abnormality to define, classify, and study. Prognosis of heterotaxy is variable but most correlated with cardiac anatomy. Management is concentrated on numerous cardiac operations and requires a multidisciplinary approach to address coexisting congenital anomalies.

Diagnosis and outcome of pregnancies with prenatally diagnosed fetal dextrocardia.

OBJECTIVE: To evaluate the incidence, associated cardiac and extracardiac malformations and clinical outcome of fetuses with dextrocardia. METHOD: A retrospective review of 3556 fetal echocardiograms between 2000 and 2011 revealed 39 cases of dextrocardia. Dextrocardia was defined as right-sided positioning of the fetal heart. Prenatal and postnatal records of the fetuses were reviewed. RESULTS: The incidence was 1.1%. Of the 39 fetuses, 22 were primary dextrocardia and 17 were dextroposition. Diaphragmatic hernia was the most common cause of dextroposition with the incidence of 76%. Of the fetuses with dextroposition 35.5% had a cardiac anomaly. The survival rate of dextroposition was 31.2% and none of the survivors had an associated cardiac anomaly. Primary fetal dextrocardia was most common with situs solitus (45.4%), followed by situs ambiguous (36.3%) and then situs inversus totalis (18.1%). Structural cardiac malformations were found in 100%, 80% and 25% of fetuses with situs ambiguous, solitus and inversus, respectively. Of the dextroposition, 47.6% terminated pregnancy, 14.2% resulted in intrauterine death, 9.5% died after birth, and 28.5% survived. CONCLUSION: A wide spectrum of complex cardiac malformations are associated with fetal dextrocardia. Fetal echocardiography enables detection of complex cardiac anomalies so that parents can be appropriately counselled.

Incidental finding of complete situs inversus in a polytraumatized adult.

BACKGROUND: Situs inversus is a rare abnormality typically posing a diagnostic dilemma during routine evaluation of acute abdominal emergencies such as in acute appendicitis and cholecystitis. It is rare to detect such in the setting of trauma. OBJECTIVE: To report an incidental finding of complete situs inversus in a poly-traumatized adult. METHODS: The clinical records of the patient including preoperative evaluation, intra-operative findings and postoperative care were reviewed. RESULT: A 53 year old man presented with difficulty breathing, left sided chest pain, generalized abdominal pain and distension 18 hours after a vehicular road traffic accident. Examination revealed features of left sided haemothorax, absent heart sounds, generalized peritonitis and limb injuries. Plain chest radiograph confirmed left haemothorax with dextrocardia. He had a left closed thoracostomy tube drainage and exploratory laparotomy which revealed complete situs inversus of intra-abdominal organs alongside a jejunal perforation which was repaired. Postoperative recovery was uneventful. CONCLUSION: Complete situs inversus is uncommon and may not be anticipated in evaluation of trauma patients. Preoperative clinical and radiological evaluation may however be helpful in making a pre-operative diagnosis and further management.

[Scimitar syndrome in infancy]. / Síndrome de la cimitarra en edad pediátrica.

OBJECTIVE: To expose our 26 year experience in clinical management, interventional catheterization and surgical treatment of patients younger than 18 years with scimitar syndrome at the National Cardiology Institute. MATERIAL AND METHODS: We reviewed retrospectively all patients with scimitar syndrome in infancy between 1984 and 2010. Patients were divided in two groups: younger an older than one year at the time of the diagnosis. Medical records were analized, as well as chest radiography, electrocardiogram, echocardiogram and helicoidal tomography. All therapeutic procedures performed and their outcomes were analized. RESULTS: We studied 22 patients with scimitar syndrome, 20 of them with associated congenital heart disease. Congestive heart failure (p > or = 0.0001) and severe pulmonary hypertension (p > or = 0.002) were more frequent in patients younger than one year. We documented dextroposition and right lung hypoplasia in 14 patients, and aorto-pulmonary collateral arteries in 15 of them. Pulmonary hypertension was an important mortality risk factor (p > or = 0.007). CONCLUSIONS: Congestive heart failure and pulmonary hypertension are more frequent in patients younger than one year, and the former is a mortality risk factor. It is important to suspect this congenital heart disease in infants with dextroposition and congestive heart failure in order to provide an opportune treatment.

Un insólito caso de síndrome de Kartagener / An unusual case of kartagener syndrome

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Dextrocardia: practical clinical points and comments on terminology.

Dextrocardia is defined by the authors as a right-sided heart with a base-apex axis directed rightward, resulting from a variation in cardiac development, and not used as a general term indicating any heart in the right chest. Dextrocardia occurs in approximately 0.01% of live births and can be discovered in various clinical settings and at various patient ages. The authors review their experience with dextrocardia, discuss useful clinical points that aid in evaluating complex anatomy, recount the history of dextrocardia terminology, and note the current inconsistent nomenclature.

Dextrocardia and coronal alignment of thoracic curve: a population study.

The objective of this study was to evaluate the coronal alignment of the thoracic spine in persons with dextrocardia. Generally, the thoracic spine is slightly curved to the right. It has been suggested that the curve could be triggered by pulsation forces from the descending aorta. Since no population study has focused on the alignment of the thoracic spine in persons with situs inversus, dextrocardia, and right-sided descending aorta, we compared the radiographs of the thoracic spine in persons with dextrocardia to those having normal levocardia. Among 57,440 persons in a health survey, 11 cases of dextrocardia were identified through standard radiological screening. The miniature chest radiographs of eight persons were eligible for the present study. The study was carried out as a nested case-control study. Four individually matched (age, gender, and municipality) controls with levocardia were chosen for each case. Coronal alignment of the thoracic spine was analyzed without knowledge of whether the person had levo- or dextrocardia. A mild convexity to the left was found in all persons with dextrocardia and right-sided descending aorta (mean Cobb angle 6.6 degrees to the left, SD 2.9). Of the 32 normal levocardia persons, 29 displayed a convexity to the right, and the remaining three had a straight spine (mean Cobb angle 5.2 degrees to the right, SD 2.3). The difference (mean 11.8 degrees , SD 3.5) differed significantly from unity (P = 0.00003). In conclusion, it seems that a slight left convexity of the thoracic spine is frequent in dextrocardia. We assume that the effect of the repetitive pulsatile pressure of the descending thoracic aorta, and the mass effect of the heart may cause the direction of the convexity to develop opposite to the side of the aortic arch.

Rupture of an abdominal aortic aneurysm in a patient with a situs inversus totalis: report of a case.

An 80-year-old woman was admitted to our hospital complaining of severe abdominal and back pain. Computed tomography (CT) showed an 8.5 x 7.0-cm infrarenal abdominal aortic aneurysm (AAA) with a large contained retroperitoneal hematoma. Known situs inversus totalis was reconfirmed by CT. Just after the CT examination, the patient collapsed and was immediately taken to the operating room. Her hemodynamics stabilized after clamping of the descending aorta via a right anterolateral thoracotomy. A resection of the abdominal aneurysm and prosthetic graft replacement were successfully performed. To our knowledge, there has been no other report of a ruptured AAA in a patient with situs inversus totalis.

Fetal dextrocardia: diagnosis and outcome in two tertiary centres.

OBJECTIVE: To evaluate the incidence of fetal dextrocardia, associated cardiac and extracardiac malformations, and outcome. DESIGN: Retrospective echocardiographic study. SETTING: Two tertiary centres for fetal cardiology. PATIENTS: 81 consecutive fetuses with a fetal dextrocardia presenting at Guy's Hospital, London, between 1983 and 2003 and at Hôpital Robert Debré, Paris, between 1988 and 2003. Fetal dextrocardia was defined as a condition in which the major axis of the heart points to the right. RESULTS: The incidence was 0.22%. There were 38 fetuses (47%) with situs solitus (SS), 24 (30%) with situs ambiguus (SA), and 19 (23%) with situs inversus (SI). Structural cardiac malformations were found in 25 cases (66%) of SS, 23 cases (96%) of SA, and 12 cases (63%) of SI. Extracardiac malformations were identified in 12 cases (31%) of SS, in five cases (21%) of SA, and in two cases (10%) of SI. Of the 81 cases of fetal dextrocardia, there were 27 interrupted pregnancies (15 of 24 SA, 10 of 38 SS, and 2 of 19 SI), six intrauterine deaths (3 of 38 SS, 2 of 24 SA, and 1 of 19 SI), and five neonatal deaths (3 of 24 SA, 1 of 19 SI, and 1 of 38 SS). There were 43 survivors (24 of 38 SS, 15 of 19 SI, and 4 of 24 SA). CONCLUSION: The majority of fetuses with dextrocardia referred for fetal echocardiography have associated congenital heart disease. There is a broad spectrum of cardiac malformation and the incidence varies according to the atrial situs. Fetal echocardiography enables detection of complex congenital heart disease so that parents can be appropriately counselled.

Placement of a left ventricular assist device in a patient with dextrocardia.

Dextrocardia most commonly presents in the setting of situs inversus, but it may occur as an isolated anomaly with normal position of the abdominal organs. Herein we present a 54-year-old man with ischemic cardiomyopathy and dextrocardia with normal position of the abdominal organs who presented with an exacerbation of congestive heart failure requiring inotropic support as well as mechanical ventilation. An implantable, wearable left ventricular assist device was placed in this patient to allow for ambulation and eventual discharge home. The patient survived 4 months before he developed pneumonia and expired.

Diagnosis and outcome of dextrocardia diagnosed in the fetus.

A retrospective review of 5,539 fetal echocardiograms over a 22-year period revealed 85 cases of dextrocardia. In primary dextrocardia (46 cases), the incidence of situs solitus, inversus, and ambiguous, was similar and associated with a high incidence of complex cardiac malformations in situs solitus and situs ambiguous. Secondary dextrocardia (39 cases) was due to intrathoracic displacement and, when caused by diaphragmatic hernia, was associated with cardiac malformations in 31% of cases. Even in complex cases, fetal echocardiography was highly accurate; therefore, specific counseling can be given to parents.

[Abnormal positions of the heart. An analysis on 1039 cases]. / Anomalii de pozitie ale inimii. O analiza pe 1039 cazuri.

The aim was to study the impact of the cardiac malpositions into the group of the 1039 congenital heart diseases registered in the Pediatric Outpatient Department of "Sf. Spiridon" hospital. All patients were investigated noninvasively using clinical examination, electrocardiogram, routine Roentgenogram, two-dimensional and Doppler echocardiography, abdominal echography and, only in two cases, cardiac catheterisation. 23 (2.21%) from 1039 congenital heart diseases registered were cardiac malpositions: dextroposition--3 cases (13.04%), dextrocardia--7 cases (30.43%) and situs inversus--13 cases (56.52%). Most of the children were boys (65.21%), 70% from all cases coming from urban area. Only 3 children had structural cardiac anomalies: two cases with dextrocardia (one with atrial septal defect and one with atrioventricular canal) and one with situs inversus and tetralogy of Fallot, two of them suffering surgical correction. Psychological impact was the main problem of these children, especially during the adolescence, except the two cases with structural cardiac abnormalities who needed following and surgical treatment.

Case report: dextrocardia with situs inversus; two cases presenting differently.

Two patients presented to our centre with dextrocardia and complete situs inversus. The first a 35-year-old presented for the first time with respiratory symptoms but no cardiac symptoms and the second patient (14-year-old) presented with cardiac symptoms. Dextrocardia and situs inversus were confirmed by the chest radiograph, electrocardiograph, and abdominal ultrasound and computerised axial tomography in the two patients. Echocardiography established atrio-ventricular great arteries ventricular concordance and bicuspid aortic valve in the second patient that presented with cardiac symptoms.