Acute coronary syndrome in Dextrocardia.

Situs inversus with dextrocardia is both a clinical and diagnostic challenge for emergency physicians to properly identify acute coronary syndrome. While dextrocardia itself does not independently increase the risk of coronary artery disease, mirrored symptoms, including right-sided and rightward radiating chest pain in any patient with cardiac risk factors should raise suspicion for acute coronary syndrome. In patients with a reversed cardiac silhouette on a chest radiograph, a reversed electrocardiogram, to include both the precordial and limb leads, is necessary to evaluate for cardiac ischemia in presumed dextrocardia. The authors present a case of a 66-year-old man with dextrocardia who presented with shortness of breath and hypotension. Rapid application of a reversed electrocardiogram resulted in the timely diagnosis of ST-segment elevation myocardial infarction and activation of the cardiac catheterization laboratory resulting in the preservation of this patient's life.

Kartagener's syndrome: a case report.

BACKGROUND: Kartagener's syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disorder characterized by the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus. Abnormal ciliary structure or function leading to impaired ciliary motility is the main pathophysiologic problem in Kartagener's syndrome. CASE PRESENTATION: A 24-year-old man from Gondar town, North-West Ethiopia, presented to University of Gondar Hospital with recurrent episodes of nasal congestion with itching and paranasal discomfort, and productive cough for more than a decade. Clinical and imaging findings revealed chronic sinusitis, bronchiectasis, dextrocardia, and situs inversus. He was treated with orally administered antibiotics, mucolytic, and chest physiotherapy. He was symptomatically better with the above therapy, and started on a long-term low-dose prophylactic antibiotic. CONCLUSIONS: Patients with Kartagener's syndrome exist in Ethiopia as cases of chronic recurrent sinopulmonary infections. As there is no easy, reliable non-invasive diagnostic test for Kartagener's syndrome and the correct diagnosis is often delayed by years, it may cause chronic respiratory problems with reduced quality of life. Genetic counseling and fertility issues should be addressed once Kartagener's syndrome is diagnosed.

Comparing levocardia and dextrocardia in fetuses with heterotaxy syndrome: prenatal features, clinical significance and outcomes.

BACKGROUND: To investigate the differences in cardiovascular disease, extracardiac anomalies and outcomes between fetuses with levocardia and dextrocardia. METHODS: Clinical demographics, prenatal features, postnatal characteristics and the outcomes of fetuses with levocardia or dextrocardia were recorded and analyzed. RESULTS: Sixty-five fetuses with dextrocardia and thirty-eight fetuses with levocardia were enrolled. Right ventricle outlet obstruction, atrioventricular septal defect and intestinal malrotation were common in both groups. Univentricular physiology, transposition of the great arteries and esophageal atresia were more frequent in fetuses with levocardia, whereas abnormal pulmonary venous connection, double outlet of right ventricle, left ventricle outlet obstruction and brain abnormalities were more frequent in the dextrocardia group. The accuracy of evaluating cardiac malformations was high, but the sensitivity in assessing extracardiac abnormalities was low. CONCLUSIONS: Although the disorders have certain overlapping features, there are several differences between fetuses with levocardia and dextrocardia. These findings might improve patient counseling and perinatal management.

Síndrome PAGOD y anomalías vasculares: ¿es un defecto de la angiogénesis embrionaria? Un nuevo caso y revisión / PAGOD syndrome and vascular anomalies: is a defect embryonic angiogenesis? A case report and review

El Síndrome PAGOD es un acrónimo de hipoplasia de pulmón y arterias pulmonares, agonadismo, onfalocele / defecto diafragmático y dextrocardia. Se describe una serie de 21 pacientes, en la cual, 90,5 % presentó un cariotipo 46,XY y solo dos casos 46,XX; el 66,6 % exhibió un fenotipo femenino y 28,6 % genitales ambiguos. La ocurrencia de dos paciente 46,XX excluye al cromosoma Y como portador del defecto genético y plantea la posibilidad de una herencia recesiva ligada al cromosoma X, sin descartar que los casos observados en hermanos puedan deberse a mutaciones en otros genes como STRA6, VEGFA, VEGFB, VEGFC, transcritos de empalmes alternativos de VEGFA, HIF1, HIF2, entre otros. Las malformaciones congénitas observadas en los pacientes fueron: genitales y gónadas 85,7 %, diafragma y pared 66,6 %, cardíaco 80,9 %, pulmonar 71,4 %, vascular 80,9 % y abdomen 42,8 %. La revisión de los pacientes ha demostrado un alto grado de variabilidad en la expresividad de malformaciones de órganos, aparatos o sistemas. Las malformaciones vasculares representan un componente importante y característico del síndrome PAGOD y cuya base morfogenética del síndrome pueda deberse a un defecto de la angiogénesis embrionaria temprana con repercusión en la organogénesis de aparatos y sistemas. Dentro de los genes relacionados con el remodelamiento vascular durante la embriogénesis, regeneración tisular y carcinogénesis está el Factor de Crecimiento del Endotelio Vascular D (VEGFD), localizado en Xp22.31, con expresión en pulmón, corazón, intestino delgado, pulmón fetal, útero, mamas, tejido neural y neuroblastoma, el cual representa un fuerte candidato para su análisis molecular como una de las posibles causa del síndrome.

PAGOD Syndrome is an acronym for lung and pulmonary arteries hypoplasia, agonadism, omphalocele / diaphragmatic defect and dextrocardia. A series of 21 patients is described, where 90.5% had a 46,XY karyotype and only two cases 46,XX; 66.6% exhibited a female phenotype and 28.6% ambiguous genitalia. The occurrence of two patients 46,XX excludes the Y chromosome as a carrier of the genetic defect and raises the possibility of a recessive X-linked inheritance, without ruling out that the observed cases in siblings may be due to mutations in other genes as Stra6, VEGFA, VEGFB, VEGFC, and alternative splicing of transcripts VEGFA, HIF1, HIF2, among others. Congenital malformations were observed in patients’ genitals and gonads 85.7%, 66.6% in diaphragm and abdominal wall , heart 80.9%, 71.4% lungs, blood vessels 80.9% and 42.8% in abdomen. The review of patients has demonstrated a high degree of variability in the expression of malformations of organs and organ systems. Vascular malformations represent an important and characteristic component of PAGOD syndrome and whose base morphogenetic syndrome may be due to a defect in early embryonic angiogenesis with impact on organogenesis and system development. Among genes related to vascular remodeling during embryogenesis, tissue regeneration and carcinogenesis, the Endothelial Growth Factor D Vascular (VEGFD), located in the Xp22.31 region, with expression in lung, heart, small intestine, uterus, breast, neuroblastoma and neural tissue, represents a strong candidate for molecular analysis as a cause of the syndrome.

[An older woman with dextrocardia and shortness of breath]. / Een oudere vrouw met dextrocardie en kortademigheid.

A 73-year-old woman, who had a history of chronic sinusitis, presented to the emergency department with shortness of breath. Additional imaging demonstrated situs inversus totalis and multiple bronchiectases. The clinical triad of sinusitis, situs inversus totalis and bronchiectasis is often characteristic of Kartagener's syndrome, a subset of primary ciliary dyskinesia.

PAGOD syndrome and vascular anomalies: is a defect embryonic angiogenesis? A case report and review.

PAGOD Syndrome is an acronym for lung and pulmonary arteries hypoplasia, agonadism, omphalocele / diaphragmatic defect and dextrocardia. A series of 21 patients is described, where 90.5% had a 46,XY karyotype and only two cases 46,XX; 66.6% exhibited a female phenotype and 28.6% ambiguous genitalia. The occurrence of two patients 46,XX excludes the Y chromosome as a carrier of the genetic defect and raises the possibility of a recessive X-linked inheritance, without ruling out that the observed cases in siblings may be due to mutations in other genes as Stra6, VEGFA, VEGFB, VEGFC, and alternative splicing of transcripts VEGFA, HIF1, HIF2, among others. Congenital malformations were observed in patients' genitals and gonads 85.7%, 66.6% in diaphragm and abdominal wall , heart 80.9%, 71.4% lungs, blood vessels 80.9% and 42.8% in abdomen. The review of patients has demonstrated a high degree of variability in the expression of malformations of organs and organ systems. Vascular malformations represent an important and characteristic component of PAGOD syndrome and whose base morphogenetic syndrome may be due to a defect in early embryonic angiogenesis with impact on organogenesis and system development. Among genes related to vascular remodeling during embryogenesis, tissue regeneration and carcinogenesis, the Endothelial Growth Factor D Vascular (VEGFD), located in the Xp22.31 region, with expression in lung, heart, small intestine, uterus, breast, neuroblastoma and neural tissue, represents a strong candidate for molecular analysis as a cause of the syndrome.

Síndrome de Poland. A propósito de un caso y revisión de la literatura / Poland syndrome. Case report and review of the literature

El síndrome de Poland es una deformidad congénita poco frecuente, esporádica y de patogenia incierta. Se caracteriza por ausencia/hipoplasia del pectoral mayor, alteraciones de la mano y de la glándula mamaria ipsolateral. Se presenta un caso de un varón con clínica sugestiva de síndrome de Poland en el hemitórax izquierdo, sin alteración de la extremidad superior, aunque asociaba dextrocardia y herniación pulmonar, una relación poco frecuente. Se hace hincapié en la necesidad de contar con un equipo multidisciplinario para su manejo inicial y tratamiento a largo plazo (AU)

The Poland syndrome is a rare, sporadic and congenital deformity with uncertain pathogenesis. It is characterized by absence or hypoplasia of the pectoralis major muscle, malformations of the hand and involvement of the ipsilateral mammary gland. A case of a newborn with clinical manifestations suggestive of Poland syndrome on the left hemithorax, associated dextrocardia and defect pulmonary despite being a rare association. Emphasis on the need for a multidisciplinary team in the initial management and long-term treatment (AU)

A Temporal Correlation Between Electrocardiogram and Computed Tomography in Acquired Postpneumonectomy Dextrocardia.

Herein, we describe a temporal correlation between the electrocardiographic changes and the chest computed tomographic findings in a 73-year-old woman who underwent a right pneumonectomy (RP) for lung adenocarcinoma.

A unique variant of Poland-Mobius syndrome with dextrocardia and a 3q23 gain.

The combined Poland and Mobius syndrome occurs rarely and with a wide range of features. There is no consensus on the etiology of this syndrome; familial, sporadic cases and likely environmental insult cases have been reported. This sporadic case represents a unique variant in the spectrum of this syndrome.

Mitral valve surgery in a patient with dextrocardia and 180° counter-clockwise rotated heart due to congenital agenesis of the right lung.

We report a case of severe mitral regurgitation (MR) with dextrocardia and 180° counterclockwise rotated situs solitus heart. We describe the technique for mitral valve surgery in a patient with dextrocardia and agenesis of the right lung.

Unilateral pulmonary hypoplasia with abdominal situs inversus.

A case of unilateral pulmonary hypoplasia in association with abdominal situs inversus (Situs Inversus Partialis) is described here in a 2-mo-old baby. The normally related heart (levocardia) is dextroposed due to the hypoplastic right lung and compensatory hyper-inflation of the opposite lung which clinically mimicked a mirror-image situs inversus totalis. Such a combination, to the best of authors' knowledge, has never been reported in the world literature.

Poland syndrome associated with ipsilateral lipoma and dextrocardia.

A 22-year-old man was admitted with a rapidly enlarging soft mass on the left chest wall, which was diagnosed as lipoma by postoperative pathology. A chest roentgenogram revealed a defect of the fourth rib, scoliosis, dextrocardia, and diaphragmatic hernia. A computed tomographic scan showed maldevelopment of the pectoralis major and minor muscles. This is the first reported case of Poland syndrome with ipsilateral lipoma of the chest wall. Dextrocardia associated with Poland syndrome may be considered dextroposition, rather than a dextroinversion, and it may arise as a result of Poland syndrome.

Combined spontaneous contralateral pneumothorax and post-pneumonectomy mediastinal shift-associated dextrocardia.

We report here on an unusual late postoperative presentation of extreme post-pneumonectomy dextrocardia and spontaneous contralateral pneumothorax presenting as late complications occurring approximately 2 years after right-sided pneumonectomy. Computed tomography is the diagnostic modality of choice to obtain information on anatomical changes within the post-pneumonectomy space. Knowledge of the spectrum of cardiopulmonary, pleural, and other complications after lung resection is important to properly manage complications in post-pneumonectomy patients.