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.
Adult* ; Arrhythmias, Cardiac ; Bronchi ; Catheter Ablation ; Dextrocardia ; Electric Countershock ; Electrocardiography ; Hemodynamics ; Humans ; Middle Aged ; Pulmonary Artery ; Radiography, Thoracic ; Tachycardia ; Tachycardia, Supraventricular ; Thorax ; Vena Cava, Superior ; Wolff-Parkinson-White Syndrome*

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.
Amniotic Fluid ; Apgar Score ; Asphyxia ; Blood Gas Analysis ; Capillaries ; Delivery Rooms ; Dextrocardia ; Epinephrine ; Female ; Gestational Age ; Hernias, Diaphragmatic, Congenital ; Humans ; Hydrogen-Ion Concentration ; Hypertension, Pulmonary* ; Infant, Newborn* ; Lung Diseases* ; Lung* ; Meconium ; Meconium Aspiration Syndrome ; Medical Records ; Parturition ; Positive-Pressure Respiration

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.
Aged ; Atrial Appendage ; Bundle of His ; Catheters ; Dextrocardia* ; Electrocardiography ; Electrodes ; Female ; Fluoroscopy ; Heart ; Heart Atria ; Humans ; Pacemaker, Artificial ; Situs Inversus*

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.
Aneurysm ; Aortic Valve ; Aortic Valve Stenosis ; Bicuspid ; Cardiomyopathies ; Dextrocardia ; Echocardiography ; Female ; Foramen Ovale, Patent ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Medical Records ; Mitral Valve Prolapse ; Retrospective Studies* ; 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.
Aneurysm ; Aortic Valve ; Aortic Valve Stenosis ; Bicuspid ; Cardiomyopathies ; Dextrocardia ; Echocardiography ; Female ; Foramen Ovale, Patent ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Medical Records ; Mitral Valve Prolapse ; Retrospective Studies* ; Scoliosis*

We report the case of a 37-year-old man who suffered from biventricular failure due to left isomerism, inferior vena cava interruption with azygos vein continuation, bilateral superior vena cava, double outlet of right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and isolated dextrocardia. Heart transplantation in patients with systemic venous anomalies often requires the correction and reconstruction of the upper & lower venous drainage. We present a case of heart transplantation in a patient with left isomerism, highlighting technical modifications to the procedure, including the unifocalization of the caval veins and reconstruction with patch augmentation.
Adult ; Azygos Vein ; Dextrocardia ; Drainage ; Heart Defects, Congenital ; Heart Transplantation* ; Heart Ventricles ; Heart* ; Humans ; Isomerism* ; Pulmonary Valve Stenosis ; Veins ; Vena Cava, Inferior ; Vena Cava, Superior

Most of the congenital diaphragmatic hernia (CDH) cases are diagnosed at prenatal period or immediately after birth with severe respiratory symptom. The classic triad, which is respiratory distress, apparent dextrocardia and a scaphoid abdomen, is usually seen in this period. Several case reports have described older infants and children with a wide spectrum of symptoms of CDH, whereas extremely few cases were reported in neonatal period except classic triad such as straungulation of the bowel. These atypical manifestations can lead physician to delayed diagnosis. We report two cases of CDH newborns. First case was diagnosed with pneumoperitoneum following tension pneumothorax, transient diaphragm eventration on 5 days after birth. The other case was diagnosed with failure to thrive and mediastinal mass on 30 days after birth. These cases suggest physicians to consider CDH in late newborn period with pneumoperitoneum following tension pneumothorax, transient diaphragm eventration, failure to thrive, and mediastinal mass.
Abdomen ; Child ; Delayed Diagnosis ; Dextrocardia ; Diaphragmatic Eventration ; Failure to Thrive ; Hernia, Diaphragmatic* ; Humans ; Infant ; Infant, Newborn* ; Parturition ; Pneumoperitoneum ; Pneumothorax

Aged ; Angioplasty, Balloon, Coronary ; Anterior Wall Myocardial Infarction ; complications ; therapy ; Dextrocardia ; complications ; Female ; Humans ; Situs Inversus ; complications ; Stents

Situs inversus totalis is a very rare anatomical abnormality, which is characterized by dextrocardia and "mirror image" distribution of abdominal organs. Dextrocardia and situs inversus have important surgical implications due to altered anatomy. The aim of this study was to describe the anatomical structures of the situs inversus with dextrocardia in Korean. Presence of situs inversus with dextrocardia was confirmed in a 72-year-old female cadaver during dissection in a gross anatomy course. It was observed by focusing on the positions of the thoracoabdominal structures. In this cadaver, clearly inverted morpholoiges existed in the structures of the thoracoabdomen except the kidneys. The left-sided kidney was located at a slightly higher level than the right-sided kidney although the liver was located on the left side. We reported herein a case of dextrocardia and situs inversus with incomplete inversion, which will provide useful information for accurate diagnosis and relevant surgical procedures.
Aged ; Cadaver ; Dextrocardia* ; Diagnosis ; Female ; Humans ; Kidney ; Liver ; Situs Inversus*

Kartagener syndrome is characterized by the triad of situs inversus, bronchiectasis, and chronic paranasal sinusitis. Recurrent sinopulmonary infection, the major determinant for diagnosing immunodeficiency, is the most common clinical manifestation of the disease. A 17-year-old female patient presented with dyspnea, cough, sputum, nasal congestion, and rhinorrhea for more than 5 years. Nasal symptoms and dyspnea had not been controlled by intermittent treatment with mucolytics and antibiotics from primary clinics since 3 months before visiting our clinic. Chest X-ray and computed tomography showed situs inversus, dextrocardia and bronchiectasis. Paranasal sinus series revealed mucosal thickening and haziness on both maxillary sinus. Serum immunoglobulin (Ig) G4 was decreased, but total IgG was within normal range. Under the diagnosis of Kartagener syndrome with IgG4 deficiency, monthly intravenous IgG (IVIG) treatment was performed for 6 months. Her symptoms were well controlled and the frequency of antibiotics use was markedly decreased. We report a patient having the Kartagener syndrome with IgG4 deficiency that was successfully controlled with a 6-month-treatment of IVIG.
Adolescent ; Anti-Bacterial Agents ; Bronchiectasis ; Cough ; Dextrocardia ; Dyspnea ; Estrogens, Conjugated (USP) ; Expectorants ; Female ; Humans ; Immunoglobulin G ; Immunoglobulins ; Immunoglobulins, Intravenous ; Kartagener Syndrome ; Maxillary Sinus ; Reference Values ; Sinusitis ; Situs Inversus ; Sputum ; Thorax