Complete cleft sternum and congenital heart disease: Review of the literature (original) (raw)

Archives of Clinical and Medical Case Reports 853 Case Report Superior Sternal Cleft -A Rare Congenital Anomaly Associated with Atrial Septal Defect

The sternal cleft is a rare anomaly occurring in 1 in 50, 000 -100, 000 births. It results from either partial or total failure of sternal fusion early in embryological development. A few causes such as riboflavin deficiency, alcohol abuse during pregnancy, and disruption in the HOX-B4 gene have been postulated. Although asymptomatic, clinical diagnosis is easily made at birth due to the obvious chest defect and abnormal movements of the thorax. Surgery is indicated to prevent mediastinal injury, respiratory and hemodynamic sequelae, allow optimal growth of the chest, and cosmesis. We report a case of superior sternal cleft and skin defect associated with isolated ostium secundum type of atrial septal defect in an apparently healthy term female newborn. This is to create awareness among Paediatricians on this very rare type of congenital anomaly that should warrant further investigation for aetiologies and full evaluation in Nigeria. Also, to share the knowledge that will aid counseling of affected families because of possible environmental/social causes, and concerning recurrence in other siblings due to a genetic predisposition.

Superior Sternal Cleft – A Rare Congenital Anomaly Associated with Atrial Septal Defect

The sternal cleft is a rare anomaly occurring in 1 in 50, 000-100, 000 births. It results from either partial or total failure of sternal fusion early in embryological development. A few causes such as riboflavin deficiency, alcohol abuse during pregnancy, and disruption in the HOX-B4 gene have been postulated. Although asymptomatic, clinical diagnosis is easily made at birth due to the obvious chest defect and abnormal movements of the thorax. Surgery is indicated to prevent mediastinal injury, respiratory and hemodynamic sequelae, allow optimal growth of the chest, and cosmesis. We report a case of superior sternal cleft and skin defect associated with isolated ostium secundum type of atrial septal defect in an apparently healthy term female newborn. This is to create awareness among Paediatricians on this very rare type of congenital anomaly that should warrant further investigation for aetiologies and full evaluation in Nigeria. Also, to share the knowledge that will aid counseling of affected families because of possible environmental/social causes, and concerning recurrence in other siblings due to a genetic predisposition.

Complex Heart Malformation in a Six Weeks Child . Case Report

2018

The aim of our study is to present the case of a six weeks old infant, prematurely born, who was admitted to our hospital with systolic murmur and cyanosis. The diagnosis of cor triatriatum sinister associated with patent foramen ovale has been established by ultrasound examination. The infant died a month after admission. The necropsy confirmed the clinical diagnosis and also revealed the hypertrophy and dilation of the right heart, left ventricle hypoplasia, interstitial pneumonia and amniotic fluid aspiration bronchopneumonia. The possible cause of death in this case was right heart failure following pulmonary hypertension associated with acute respiratory failure. To our knowledge it is the first case with such a complex malformation reported in a premature neonate in our hospital.

An unusual presentation of congenital heart disease

Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital, 2003

We describe an unusual presentation of congenital heart disease mimicking preeclampsia in a young, gravid girl. The diagnosis of Shone's complex was confirmed by echocardiography. This complex is manifested by multiple levels of obstruction involving the left side of the heart and the systemic circulation. It is extremely rare, especially in pregnant adolescents. We briefly describe the patient's clinical history, physical examination, and treatment, as well as our clinical decisions regarding her case. The discussion focuses on the spectrum of findings in Shone's complex and the physiologic impact of therapy on this patient.

A newborn with complete heart block and structural cardiac defects

Annals of Allergy, Asthma & Immunology, 2005

HISTORY OF PRESENT ILLNESS A 3,200-g girl was born to a 37-year-old human immunodeficiency virus-negative woman at 36 weeks' gestation. The pregnancy was complicated by maternal type 2 diabetes mellitus (requiring insulin therapy during pregnancy) and by hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. 1 The baby was delivered by emergency cesarean section for maternal preeclampsia. Apgar scores were 8 at 1 minute of life and 9 at 5 minutes of life. Complex congenital heart disease had been diagnosed in utero by fetal ultrasound. The infant was promptly administered prostaglandin E 2 shortly after birth and then was transferred to the neonatal intensive care unit. A chest radiograph (Fig 1) revealed a boot-shaped heart secondary to right ventricular hypertrophy and an abnormal main pulmonary artery segment. Also notable is the absence of a thymic shadow. Echocardiography revealed complete atrioventricular heart block, pulmonary atresia, and an unbalanced atrioventricular canal. Laboratory evaluation revealed a white blood cell count of 15,700/L (reference range, 5,000-20,000/L), a hemoglobin level of 17.3 g/dL (reference range, 13.5-20.5 g/dL), a platelet count of 151,000/L (reference range, 140,000-440,000/L), an absolute lymphocyte count of 1,300/L (reference range, 2,000-17,000/L), and an absolute neutrophil count of 12,500/L (reference range, 1,000-9,500/L). Serum electrolyte values were normal except for a total calcium level of 7.5 mg/dL (reference range, 8.8-10.0 mg/ dL) and an ionized calcium level of 0.83 mmol/L (reference range, 1.03-1.23 mmol/L). The infant began peripheral hyperalimentation and underwent a Blalock-Taussig shunt and placement of an epicardial pacing system. Unifocalization of the pulmonary artery, a surgical technique to connect all pulmonary segments to convergent central pulmonary arteries, was performed on day 4 of life. It was confirmed at surgery that the thymus was

Complex Congenital Heart Disease in an Infant - A Case Report and Review

Journal of Evolution of Medical and Dental Sciences

Congenital heart disease (CHD) often poses a great diagnostic challenge for physicians. Despite antenatal diagnostic tests advancing to a great level, accurate diagnosis and treatment of congenital heart diseases is mandatory. These diseases range from mild to severe life-threatening scenarios sometimes having vague presentations making diagnosis even more difficult. Early diagnosis and treatment are usually lifesaving.1 Congenital heart diseases can often be classified as cyanotic and acyanotic based on clinical presentation. After birth, fetal structures like foramen ovale, ductus venosus and ductus arteriosus are no longer required for survival and they begin to close.2 Persistence of such structures after birth is a sign of congenital heart diseases. High mortality contributing diseases which require prompt intervention include hypoplastic left heart syndrome (HLHS), coarctation of aorta (COA), interrupted aortic arch (IAA), transposition of the great arteries (TGA), total anoma...

A patient with severe congenital pulmonary stenosis and severe right ventricular hypertrophy

Anadolu Kardiyoloji Dergisi/The Anatolian Journal of Cardiology, 2012

narrow-QRS complex tachycardia with a rate of 190 bpm . Sinus rhythm was achieved after i.v. administration of verapamil which showed 0.5-1 mm ST segment elevation in septal leads (V1-V3). Chest X-ray revealed normal findings. Transthoracic echocardiography revealed left ventricular (LV) ejection fraction of 65%, LV end-diastolic diameter of 45 mm and cystic appearance at mid segment of the interventricular septum with 19x15 mm in diameter , Video 1. See corresponding video/movie images at www.anakarder.com). Cardiac magnetic resonance imaging demonstrated a cystic lesion, 20x13 mm in size, in the left ventricular side of interventricular septum, protruding into the lumen. The cystic lesion was hypointense on T1A sequences and hyperintense on T1 and T2A images, but was not suppressed on fat suppression sequences, which was compatible with cardiac hydatid cyst . Cranial, thoracic and abdominal tomographic imaging showed no lesions of hydatid cyst. Preoperative coronary angiography revealed normal coronary arteries. Leukocyte count was 8400/mm 3 (1.2% eosinophils). However, serological findings with indirect hemagglutination test were negative for echinococcal disease. Albendazole was initiated preoperatively for three weeks. The patient was operated with right ventriculotomy and cyst excision was performed with no complication. Pathological examination also confirmed the diagnosis of hydatid cyst. The patient was well at 3rd month control without any palpitation. Echocardiography revealed no defect or lesion at the interventricular septum. Additionally, 24-h Holter monitoring revealed sinus rhythm without any conduction blocks or dysrhythmia.

Cardiac defect and extracardiac anomalies

Materia Medica, 2011

The overall survival rate in infants affected by congenital heart disease remains low. The poor outcome may be related to the increased association with extracardiac anomalies and aneupoloidy. since most types of CHd are now amenable to surgery, it is essential that any fetus with congenital structural heart defect should be completely evaluated, noting in particular congenital extracardiac defects which may adversely affect the prognosis. Noncardiac malformations have been reported to occur in up to 50% of patients with congenital heart disease. The combination of cardiac anomalies with other organ abnormalities appears in genetic syndromes and after exposure to environmental factors. Fetuses with prenatally diagnosed cardiac malformations need to have offer structural abnormalities ruled out. They should be evaluated thoroughly by ultrasound exam, because the detection of an extracardiac anomaly may dramatically affect prognosis. also, fetuses in which an extracardiac anomaly or chromosome abnormality is diagnosed deserve a complete evolution with full fetal echocardiography. Fetal caryotype analysis should be performed since the risk of aneuploidy approaches 40% in cases of prenatally diagnosed CHd.