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

Kartagener's syndrome: a case report

Kartagener's syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disease, and is characterized by the triad of chronic sinusitis, bronchiectasis, and situs inversus. This paper reports the case of a 27-year-old female presenting with dyspnea on medium exertion, accompanied by chronic cough, non-productive or with clear expectoration. She had recurrent pneumonia until 15 years of age and underwent a lobectomy in the lower lobe of the left lung, probably due to bronchiectasis. Chest computed tomography showed situs inversus totalis, signs of previous surgical manipulation, and mild bronchial thickening. Computed tomography of the paranasal sinuses showed signs of chronic sinusitis due to a probable ciliary kinesis disorder. These finding suggest the diagnosis of Kartagener's syndrome. The prognosis reveals a slow rate of decline in lung function. However, repeated or chronic infections can negatively influence the quality of life of these patients.

Dextrocardia con doble salida del ventrículo derecho con comunicación interventricular no relacionada asociada a malposición de grandes vasos / Right ventricular double outlet dextrocardia with unrelated ventricular septal defect associated with great vessel malposition

Resumen En el siguiente reporte se expone un caso complejo en el que coexisten malformaciones cardiacas congénitas de presentación atípica con un doble tracto de salida del ventrículo derecho, en un paciente con múltiples anomalías menores en la exploración física y un cariotipo normal. La atipia de cada una de estas y su coexistencia hacen pensar en posibles alteraciones genéticas que aún son desconocidas. Lo anterior supone un reto terapéutico con el fin de restaurar una fisiología cardiaca compatible con la vida, lo cual se logra en este caso mediante un cerclaje exitoso de la arteria pulmonar.

Abstract The following report presents a complex case in which congenital cardiac malformations of atypical presentation coexist with a double outlet right ventricle, in a patient with multiple minor abnormalities on the physical examination and a normal karyotype. The atypia of these and their coexistence suggest possible genetic alterations that are still unknown. Therefore, a therapeutic challenge in order to restore a cardiac physiology compatible with life is proposed, which is achieved in this case through a successful banding of the pulmonary artery.

Pseudodextrocardia: una nueva defifinición / Pseudo-dextrocardia: a new defifinition

La pseudodextrocardia se ha definido como un desplazamiento irregular del corazón y sus estructuras vasculares hacia la derecha por causas extracardiacas. Es un hallazgo ocasional en estudios imagenológicos de tórax o abdomen y varía acorde con las diferentes relaciones causales. Se presenta el caso de un paciente con una neumopatía crónica, quien a su ingreso presenta pseudodextrocardia como hallazgo incidental.

Pseudo-dextrocardia has been defined as an unusual displacement of the heart and its vascular structures to the right secondary to extracardiac causes. It is an occasional finding in thoracic or abdominal imaging studies and varies according to the various causal relationships. We report a case of pseudo-dextrocardia as an incidental finding in a patient admitted with chronic pulmonary disease.

Dextrocardia como hallazgo incidental: revisión a partir de un caso / Dextrocardia as incidental finding: review from a case

INTRODUCTION: Dextrocardia is characterized by the positioning of the heart to the right of the thoracic cavity, usually with the apex oriented to the right and its inverse anatomy 1,2. It is a rare congenital pathology that can be associated with other congenital cardiac anomalies, as well as a change in position of all thoracoabdominal structures 1-4. Its diagnosis in adult life is usually incidental. We present the clinical case of a 64-year-old man with an electrocardiogram that showed deviation of the QRS complex axis to the extreme right and a physical examination compatible with dextrocardia, which was later confirmed with images. OBJECTIVES: To review the medical literature related to the diagnosis of dextrocardia through the presentation of a clinical case. METHODS: The clinical data of the case were collected by means of anamnesis, physical examination and specialized exams of the patient, as well as his clinical file. The literary review was made using the MEDLINE® search engine for scientific journals. RESULTS: Dextrocardia affects less than 1% of the general population according to the studies reviewed. Its importance lies in the association with other congenital pathologies such as cardiac and extracardiac structural malformations, which increases morbidity and mortality in this group of patients. It is imperative, when suspected, complete the study with images that allow confirming or ruling out other structural anomalies.

Situs inversus totalis asociado a síndrome de preexcitación ventricular: reporte de un caso / Situs inversus totalis and pre excitation syndrome

Resumen: El situs inversus totalis es la inversión congénita completa de órganos torácicos y abdominales. Se presenta el caso de una paciente de 3 años sin antecedentes médicos previos, a quien en atención primaria, y por un cuadro respiratorio agudo, se evidencia el hallazgo de dextrocardia y burbuja gástrica a derecha en la radiografía de tórax, sospechándose situs inversus totalis. Fue derivada a cardiología infantil donde se confirmó el diagnóstico con un ecocardiograma transtorácico, asociado a un hallazgo de preexcitación ventricular en el electrocardiograma. Una vez resuelto el cuadro respiratorio agudo, la paciente se mantiene controlada de manera periódica en atención primaria y por especialista de manera semestral.

Abstract: Situs inversus totalis is the complete congenital inversion of thoracic and abdominal organs. We present the case of a 3-year-old girl with no previous medical history. When seen with an acute respiratory syndrome, dextrocardia and gastric bubble on the right side led to the diagnosis of Situs inversus. She was referred to infant cardiology where the diagnosis was confirmed with a transthoracic echocardiogram. In addition, the electrocardiogram identified the presence of ventricular preexitation. Once the acute respiratory symptoms subsided, the patient remains controlled periodically in primary care and by a specialist every six months. No episodes of tachycardia have been detected.

Dextrocardia and situs inversus totalis in a Turkish subject: a case report / Dextrocardia y situs inversus totalis en un sujeto turco: reporte de un caso

Dextrocardia with situs inversus is an uncommon anomaly affecting about 1 to 2 per 10,000 in the general population. This report describes an adult male patient with dextrocardia and in a Turkish subject. The photographic illustrations revealed transposition of some of the visceral organs such as the spleen was located right and the liver and gall bladder on the left. The heart was flattened and flipped to the right. Many people with situs inversus totalis are unaware of their unusual anatomy until they seek medical attention for an unrelated condition. So, early detection may lead to a successful surgical management and consequently offer a safer chance of survival. This report showed that dextrocardia and situs inversus can be seen amongst Turkish subjects.

La dextrocardia con situs inversus es una anomalía poco frecuente que afecta aproximadamente de 1 a 2 personas por 10.000 en la población general. Este informe describe un paciente masculino adulto con dextrocardia. Las figuras revelaron que la transposición de algunos de los órganos viscerales, como el bazo, se ubicada a la derecha y el hígado y la vesícula biliar a la izquierda. El corazón fue aplastado y girado hacia la derecha. Muchas personas con situs inversus totalis desconocen su anatomía inusual hasta que buscan atención médica por una afección no relacionada. Por lo tanto, la detección temprana puede llevar a un manejo quirúrgico exitoso y, en consecuencia, ofrecer una posibilidad más segura de supervivencia. Este informe mostró que la dextrocardia y el situs inversus se pueden encontrar entre los sujetos turcos.

Caso Clínico: Discinesia Ciliar Primaria (Síndrome de Kartagener) / Clinical Case Report: Primary Ciliary Dyskinesia (Kartagener's Syndrom

INTRODUCCIÓN: La discinesia ciliar primaria es un trastorno hereditario autosómico recesivo, que afecta la función de las células ciliadas y se caracteriza por infecciones respiratorias a repetición y afecta tanto al tracto respiratorio superior e inferior, puede asociarse con trastornos de la lateralidad orgánica (síndrome de Kartagener), infertilidad y en algunos casos malformaciones. No existe un tratamiento específico; sin embargo, se tratan las infecciones agudas y se realiza seguimiento de la función pulmonar como en el caso clínico que se presenta a continuación. CASO CLÍNICO: Se trata de una mujer de 28 años, con antecedentes de dextrocardia, sinusitis, otitis, bronquitis y neumonías a repetición, asmática, con rinorrea mucoide crónica, que acudió por cuadro persistente de tos productiva y disnea de moderados esfuerzos. Al examen físico destacó: saturación de 80% con FIO2: 21%, cianosis discreta, ruidos cardiacos audibles en hemitórax derecho con reforzamiento del segundo ruido, estertores difusos y frémito aumentado. En la espirometría se detectó patrón obstructivo ­ restrictivo severo, la tomografía demostró la presencia de sinusitis maxilar y esfenoidal, dextrocardia, bronquiectasias e infiltrados difusos, poliesplenia, hepatomegalia e hígado en herradura. Se diagnosticó de síndrome de Kartagener (por dextrocardia, sinusitis y bronquiectasias). EVOLUCIÓN: Durante la estancia hospitalaria la paciente permaneció sin requerimientos de oxígeno suplementario y afebril. Recibió tratamiento antibiótico, corticoides inhalatorios y salbutamol. Se explicó a la paciente y sus familiares la benignidad de la enfermedad y el requerimiento de controles rigurosos por consulta externa. El diagnóstico definitivo por microscopía electrónica no fue realizado por falta de recursos a nivel local. CONCLUSIÓN: La discinesia ciliar primaria por lo general tiene un curso evolutivo de carácter benigno, al ser una enfermedad poco conocida su diagnóstico es tardío. La discinesia ciliar primaria debe ser considera dentro de los diagnósticos diferenciales de un paciente que presenta infecciones respiratorias a repetición.(au)

BACKGROUND: Primary ciliary dyskinesia is an inherited autosomal recessive disorder, which affects the function of ciliated cells and is characterized by recurrent upper and lower respiratory infections. It may be associated with organic laterality disorders (Kartagener syndrome), infertility and in some cases malformations. There is no specific treatment; however, acute infections management and pulmonary function surveillance is recommended, as presented in the case report. CASE REPORT: 28-year-old woman with a history of dextrocardia, sinusitis, otitis, bronchitis and recurrent pneumonia, asthmatic, with chronic mucoid rhinorrhea and recurrent episodes of productive cough and dyspnea. Physical examination revealed an oxygen saturation of 80% at room air, discrete cyanosis, and audible cardiac sounds in the right hemithorax with reinforcement of the second noise, diffuse rales and increased thrill. Pulmonary function test was positive for a severe obstructive - restrictive pattern, computed tomography revealed the presence of maxillary and sphenoid sinusitis, dextrocardia, bronchiectasis, polysplenia hepatomegaly and horseshoe liver. The diagnosis of Kartagener syndrome was made (due to dextrocardia, sinusitis and bronchiectasis). EVOLUTION: During the hospital stay the patient remained without oxygen requirements, she received antibiotic treatment plus corticosteroids and salbutamol. Patient education was carried out, indicating the benignity of the disease and the requirement of close monitoring. Definitive diagnosis by electron microscopy was not available. CONCLUSION: Primary ciliary dyskinesia usually has a benign course of evolution; being an uncommon disease, diagnosis is usually late. Primary ciliary dyskinesia should be considered within the differential diagnosis of patients with recurrent respiratory infection(au)

Síndrome de Kartagener: reporte de caso / Kartagener syndrome: case report

El Síndrome de Kartagener es una enfermedad autosómica recesiva, caracterizada por discinesia ciliar primaria la cual consiste en la disfunción de las células ciliadas. Esta es la causa de la manifestación de la sintomatología respiratoria que presenta este síndrome: tos, sinusitis, otitis media y bronquiectasias. Otra sintomatología que acompaña a este síndrome son infertilidad y situs inversus. El reconocimiento de esta enfermedad data desde los principios del siglo XX y se constituye en síndrome gracias a la descripción de 4 casos clínicos, por Manes Kartagener, neumólogo que trabajaba en Zurich, quien describió por primera vez en 1933 la triada característica de este síndrome: sinusitis crónica, bronquiectasias y situs inversus. Paciente femenina de 35 años con antecedentes de infecciones del tracto respiratorio superior e inferior a repetición desde la infancia, se presenta a la emergencia del Hospital Mario Catarino Rivas con un episodio infeccioso grave de las vías respiratorias. Durante su estadía hospitalaria se confirma dextrocardia y situs inversus por medio de estudios radiológicos por lo que se confirma el diagnostico de Sindrome de Kartagener. El síndrome de Kartagener que se caracteriza por la triada clásica de sinusitis crónica, bronquiectasias y situs inversus. En el curso de su presentación clínica se espera encontrar infecciones a repetición del tracto respiratorio superior e inferior, gracias al compromiso de la movilidad ciliar normalmente encargada de la remoción de patógenos ambientales, a largo plazo se espera el desarrollo de las dilataciones bronquiales a consecuencia del cúmulo de material mucoide y la reacción inflamatoria local.

Kartagener's syndrome is an autosomal recessive disease, characterized by primary ciliary dyskinesia, which consists of ciliated cell dysfunction. This is the cause of the manifestation of the respiratory symptomatology that presents this syndrome-cough, sinusitis, and bronchiectasis. Another symptomatology that accompanies this syndrome are infertility and situs inversus. Its recognition from the early XX century, and constitutes syndrome thanks to the description of 4 clinical cases by Manes Kartagener, a pulmonologist who worked in Zurich and first described the triad of sinusitis, bronchiectasis and situs inversus in 1933. A 35-year-old female patient with a history of dextrocardia, repeated respiratory infections, and inadequate response to previous treatments, is presented to the Emergency Hospital Mario Catarino Rivas. Kartagener syndrome characterized by the classic triad of chronic sinusitis, bronchiectasis and situs inversus. During clinical presentation expect to find recurring upper and lower respiratory tract, thanks to the commitment of mobility infections ciliciar normally responsible for the removal of environmental pathogens, expected long-term development of bronchial dilation because of accumulation of mucoid material and local inflammatory reaction.

Comparison between Patients with Persistent Pulmonary Hypertension of Neonates Concomitant with Parenchymal Lung Disease and Idiopathic Persistent Pulmonary Hypertension of Neonates

PURPOSE: We aimed to compare the clinical characteristics between neonates with persistent pulmonary hypertension of neonates (PPHN) with parenchymal lung disease (PLD) and those with idiopathic PPHN. METHODS: We reviewed the medical records of 67 neonates with gestational ages not lesser than 34⁺⁰ weeks who were born at Inje University Sanggye Paik Hospital between June 1, 2005 and December 31, 2016. We excluded 10 neonates who presented with congenital anomalies (n=3), dextrocardia (n=1), triple X syndrome (n=1), death before treatment (n=1), neonatal asphyxia (n=2), and congenital diaphragmatic hernia (n=2). Neonates were categorized into 2 groups—PPHN with PLD (PLD group, those diagnosed with PLD such as respiratory distress syndrome or meconium aspiration syndrome, n=36) and idiopathic PPHN (idiopathic group, n=21). We compared the clinical characteristics, treatment, and laboratory findings between the groups. RESULTS: The PLD group neonates showed a greater requirement for positive pressure ventilation in the delivery room, higher frequency of meconium staining of amniotic fluid, and greater need for surfactant application than those belonging to the idiopathic group. In contrast, epinephrine use was more common in the idiopathic PPHN group than in the PLD group. The 1-minute Apgar score and pH observed on initial capillary blood gas analysis were lower in the PLD than in the idiopathic group. Severity scores were higher in the idiopathic than in the PLD group 4–7 days after birth. CONCLUSION: In our study, an overall simplified severity score in the first week after birth was higher in the idiopathic than in the PLD group. These results were particularly statistically significant over postnatal days 4–7.

A Case of Multiple Cardiovascular and Tracheal Anomalies Presented with Wolff-Parkinson-White Syndrome in a Middle-aged Adult

Congenital cardiovascular anomalies, such as dextrocardia, persistent left superior vena cava (SVC), and pulmonary artery (PA) sling, are rare disorders. These congenital anomalies can occur alone, or coincide with other congenital malformations. In the majority of cases, congenital anomalies are detected early in life by certain signs and symptoms. A 56-year-old man with no previous medical history was admitted due to recurrent wide QRS complex tachycardia with hemodynamic collapse. A chest radiograph showed dextrocardia. After synchronized cardioversion, an electrocardiogram revealed Wolff-Parkinson-White (WPW) syndrome. Persistent left SVC, PA sling, and right tracheal bronchus were also detected by a chest computed tomography (CT) scan. He was diagnosed with paroxysmal supraventricular tachycardia (PSVT) associated with WPW syndrome, and underwent radiofrequency ablation. We reported the first case of situs solitus dextrocardia coexisting with persistent left SVC, PA sling and right tracheal bronchus presented with WPW and PSVT in a middle-aged adult. In patients with a cardiovascular anomaly, clinicians should consider thorough evaluation of possibly combined cardiovascular and airway malformations and cardiac dysrhythmia.

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.

Pectus Excavatum e Situs Inversus Totalis: um relato de caso / Pectus excavatum and situs inversus totalis: a case report

O Pectus Excavatum consiste em uma deformidade óssea torácica caracterizada, basicamente, pela depressão do esterno e das cartilagens costais inferiores, podendo cursar com alterações da extremidade anterior das costelas na sua articulação com as cartilagens costais. Ademais, o Situs Inversus Totalis (STI) é caracterizado pelo desenvolvimento das vísceras no lado oposto de sua topografia habitual acompanhada de dextrocardia; ápice cardíaco voltado para a direita. Os autores relatam um caso combinado de Pectus Excavatum e Situs Inversus Totalis em um paciente jovem, do sexo masculino, diagnosticado no período da puberdade e discutem brevemente acerca de uma revisão literária do caso(AU)

Pectus excavatum is a chest bone deformity characterized primarily by depression of the sternum and the lower costal cartilages, which may be accompanied by changes in the anterior end of the ribs in its articulation with rib cartilage. In addition, the situs inversus totalis (SIT) is characterized by the development of the viscera on the opposite side of its usual topography accompanied by dextrocardia; cardiac apex facing the right. The authors report a combined case of pectus excavatum and situs inversus totalis in a young male patient diagnosed in the puberty period and briefly discuss about a literature review of the case(AU)

Off-pump triple coronary artery bypass grafting in a patient with situs inversus totalis: case presentation and a brief review of the brazilian and the international experiences

Abstract A 76-year-old man with situs inversus totalis underwent a successful off-pump three-vessel coronary artery bypass surgery. The postoperative course was uneventful, and the patient was discharged 8 days later. At 9-month follow-up a coronary computed tomography angiography confirmed the viability of all of the grafts, and one year after the operation the patient remained asymptomatic. It comprises the fifth Brazilian case of a coronary surgery in a patient with situs inversus totalis and the first one of the country of a coronary artery bypass surgery without the use of the cardiopulmonary bypass in this condition.

Permanent Pacemaker Implantation in a Patient with Mirror-Image Dextrocardia and Situs Inversus Totalis / 대한내과학회지

A 68-year-old female patient with situs inversus totalis was referred for permanent pacemaker implantation to treat a symptomatic sinus pause. The initial electrocardiographic findings suggested the presence of mirror-image dextrocardia. No congenital anomalies were evident on pre-procedural examination. To explore the relevant anatomy, electrode catheters were placed at the right ventricular apex, the His bundle area, and the high right atrium under guidance via intracardiac electrography and fluoroscopy. The left and right anterior oblique views of the dextrocardia were mirror images of the right and left (respectively) anterior oblique views of a normal heart. Pacing leads were successfully positioned at the upper interventricular septum and the right atrial appendage. Dextrocardia is a rare congenital anomaly, and the accompanying anatomical distortions can render device implantation challenging. We suggest that pre-procedural fluoroscopic evaluation using electrode catheters can provide critical data on anatomical landmarks allowing effective positioning of pacing leads.

A Retrospective Study of Congenital Cardiac Abnormality Associated with Scoliosis

STUDY DESIGN: Retrospective study. PURPOSE: To identify the incidence of congenital cardiac abnormalities in patients who had scoliosis and underwent surgical treatment for scoliosis. OVERVIEW OF LITERATURE: Congenital and idiopathic scoliosis (IS) are associated with cardiac abnormalities. We sought to establish and compare the incidence of congenital cardiac abnormalities in patients with idiopathic and congenital scoliosis (CS) who underwent surgical treatment for scoliosis. METHODS: Ninety consecutive scoliosis patients, who underwent surgical correction of scoliosis, were classified as CS (55 patients, 28 female [51%]) and IS (35 patients, 21 female [60%]). The complete data of the patients, including medical records, plain radiograph and transthoracic echocardiography were retrospectively assessed. RESULTS: We found that mitral valve prolapse was the most common cardiac abnormality in both patients with IS (nine patients, 26%) and CS (13 patients, 24%). Other congenital cardiac abnormalities were atrial septal aneurysm (23% of IS patients, 18% of CS patients), pulmonary insufficiency (20% of IS patients, 4% of CS patients), aortic insufficiency (17% of IS patients), atrial septal defect (11% of IS patients, 13% of CS patients), patent foramen ovale (15% of CS patients), dextrocardia (4% of CS patients), bicuspid aortic valve (3% of IS patients), aortic stenosis (2% of CS patients), ventricular septal defect (2% of CS patients), and cardiomyopathy (2% of CS patients). CONCLUSIONS: We determined the increased incidence of congenital cardiac abnormalities among patients with congenital and IS. Mitral valve prolapse appeared to be the most prevalent congenital cardiac abnormality in both groups.