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Advances in multidetector computed tomography (CT) technology have revolutionized cardiovascular imaging in children. Faster acquisition times and high-quality reconstructions have enabled CT to become a valuable modality for the evaluation of cardiac and vascular pathology. Identification of congenital cardiovascular anomalies and their complications is important for patient management and treatment planning. CT offers an excellent technology in this patient population. The confident diagnosis of congenital cardiovascular lesions requires awareness of the appearances of these defects on CT, before and after surgery. In addition, understanding the techniques for performing CT in the pediatric population, including the risks, is essential for the radiologist.

Objective: To investigate the prevalence and diagnostic value of first-pass myocardial perfusion defects (PD) visualised by contrast-enhanced multidetector computed tomography (MDCT) in patients admitted for a first acute coronary syndrome (ACS)., Methods: Thirty-eight patients with non-ST segment elevation myocardial infarction (NSTEMI) or unstable angina (UA) and scheduled for percutaneous coronary intervention underwent dual-source CT immediately before catheterisation. CT images were analysed for the presence of any PD by using a 17-segment model. Results were compared with peak cardiac troponin-I (cTnI) and angiography findings., Results: PD were seen in 21 of the 24 patients with NSTEMI (median peak cTnI level 7.07 ng/mL; range 0.72-37.07 ng/mL) and in 2 of 14 patients with UA. PD corresponded with the territory of the infarct-related artery in 20 out of 22 patients. In a patient-based analysis, sensitivity, specificity, negative and positive predictive values of any PD for predicting NSTEMI were 88%, 86%, 80% and 91%. Per culprit artery, the respective values were 86%, 75%, 80% and 83%., Conclusion: In patients with non-ST segment elevation ACS, first-pass myocardial PD in contrast-enhanced MDCT correlate closely with the presence of myocardial necrosis, as determined by increases in cTnI levels.

Introduction: Aberrant right subclavian artery arising from the distal part of the aortic arch and passing behind the oesophagus (arteria lusoria) is a rare congenital vascular anomaly, it is the 4th most common aortic arch anomaly. At the site of the orifice there is the Kommerell's diverticulum, which is the locus minoris resistentiae with the possible arise of the aortic aneurysm. Aneurysmatic dilatation of the anomalous artery and of the aorta may be the cause of distal embolism or rupture. Less frequently there is also the possibility of aortic dissection or traumatic rupture., Case Report: 54-old man with a symptomatic aberrant aneurysmatic dilated right subclavian artery (arteria lusoria) and an anerurysmatic dilatation of the Kommerell's diverticulum was indicated to staged combined management. Firstly we performed open surgical debranching of two supraaortic trunks (both subclavian arteries). Thereafter we excluded the orifice of the aberrant artery with the Kommerell's diverticulum by use of the endovascular techniques. There have been no complications during the perioperative period and the effect of surgery was optimal., Discussion: It is generally accepted that the presence of aneurysm of the aberrant right subclavian artery is an indication for surgery, whether symptomatic or not. The conventional surgery is usually staged. Firstly there is a carotid-subclavian bypass or transposition on the right side and thereafter a transthoracic resection of the Kommerell's diverticulum and aortic angioplasty. The combined management with supraaortic revascularization followed with the stentgraft exclusion of the aneurysm is a sophisticated alternative., Conclusion: Experiences with the combined treatment published in the literature and ours are excellent, this technique is miniinvasive with a low complication rate. In our opinion it is the management of choice.

Background: Identifying persons at risk for sudden cardiac death (SCD) is challenging. A comprehensive evaluation may reveal clues about the clinical, anatomical, genetic, and metabolic risk factors for SCD., Methods: Seventy-one patients who had SCD (25-60 years old) without an initially apparent cause of death were evaluated at the Hennepin County Medical Examiner's office (Minneapolis, MN) from August 2001 to July 2004. We reviewed their clinic records conducted next-of-kin interviews and performed autopsy, laboratory testing, and genetic analysis for mutations in genes associated with the long QT syndrome., Results: Mean age was 49.5 +/- 7 years, 86% were male, and 2 subjects had history of coronary heart disease (CHD). Coronary risk factors were highly prevalent in comparison to individuals of the same age group in this community (eg, smoking 61%, hypertension 27%, hyperlipidemia 25%) but inadequately treated. On autopsy, 80% of the subjects had high-grade coronary stenoses. Acute coronary lesions and previous silent myocardial infarction (MI) were found in 27% and 34%, respectively. Furthermore, 32% of the subjects had recently smoked cigarettes, and 50% had ingested analgesics. Possible deleterious mutations of the ion channel genes were detected in 5 subjects (7%). Of these, 4 were in the sodium channel gene SCN5A., Conclusions: Most of the persons who had SCD in the community had severe subclinical CHD, including undetected previous MI. Traditional coronary risk factors were prevalent and undertreated. Mutations in the long QT syndrome genes were detected in a few subjects. These findings imply that improvements in the detection and treatment of subclinical CHD in the community are needed to prevent SCD., (Copyright 2010 Mosby, Inc. All rights reserved.)

Purpose: : Pseudoxanthoma elasticum is an autosomal recessive disorder of elastic tissue in the skin, eyes, and cardiovascular system, caused by mutations in the ABCC6 gene. The purpose of this study was to check variability in expression within one genotype and look for pseudoxanthoma elasticum signs in heterozygotes., Methods: : We examined a relatively large, in comparison with the present literature, group of adult persons homozygous or heterozygous for the c.3775delT mutation in the ABCC6 gene, from a genetically isolated population in the Netherlands. All participants filled out a questionnaire and underwent standardized dermatologic and ophthalmologic examinations with photography of skin and fundus abnormalities. Skin biopsies from affected skin or a predilection site and/or a scar were examined and compared with biopsies from controls., Results: : Skin abnormalities, ophthalmologic signs, and cardiovascular problems varied greatly among the 15 homozygous participants. There was no correlation among severity of skin, eyes, or cardiovascular abnormalities. None of the 44 heterozygous participants had any sign of pseudoxanthoma elasticum on dermatologic, histopathologic, and/or ophthalmologic examination, but 32% had cardiovascular disease., Conclusion: : Individuals homozygous for the c.3775delT mutation can have a highly variable phenotype. We did not find pseudoxanthoma elasticum eye or skin abnormalities in the heterozygous family members.

A 57-year-old man presented with symptomatic right-to-left shunts decades after surgical closure of an atrial septal defect and partial anomalous pulmonary veins. Two sources of shunting were identified: a baffle leak from the right superior vena cava (RSVC) to the left atrium secondary to complete occlusion of the RSVC-to-right atrium connection and an acquired previously unpublished coronary sinus to the left atrial shunt secondary to inadvertent closure of the coronary sinus. The diagnosis and transcatheter management of these unusual right-to-left shunts are reported.

Objective: To analyze the cardiac manifestations of mitochondriopathy patients., Methods: We retrospectively analyzed the clinical (Electrocardiogram, Holter monitoring, echocardiogram and laboratory examinations) and pathological data of 90 mitochondriopathy patients diagnosed within recent 20 years. The cardiac involvement data from these patients were summarized., Results: Hypertrophic cardiomyopathy was found in 2 patients and dilated cardiomyopathy in 3 patients Mitochondriopathy diagnosis was made in 1 patient two years after heart transplantation due to heart failure resulting from previously diagnosed hypertrophic cardiomyopathy with noncompaction. The prevalence of cardiomyopathy is 5.6% (5/90). The prevalence of various arrhythmias was 22.2% (20/90). Four patients received permanent pacemaker because of Adams-Stokes attack or bradyarrhythmias (mitochondriopathy diagnosis was made 1-3 years post pacemaker implantation in 3 cases). History of syncope, respiratory failure, RBBB, atrial fibrillation and episodic ventricular tachyarrhythmias were presented in 1 patient with mitochondriopathy, another mitochondriopathy patient developed atrial tachyarrhythmias. Arrhythmia were present in 14 mitochondriopathy patients including RBBB, bifascicular block, intraventricular block, Wolff-Parkinson-White syndrome and short PR interval syndrome. The mtDNA 3243A-G mutation was detected in 8 patients., Conclusions: Incidence of cardiomyopathy, heart failure and severe arrhythmias is high in patients with mitochondriopathy. Therefore, young cardiomyopathy patients with severe conduction block disorders should undergo relevant etiologic and genetic screening for mitochondriopathy and patients with diagnosed mitochondriopathy should regularly receive electrocardiogram and echocardiography examinations for possible cardiac involvement.

Background: Cardiovascular diseases account for a threefold higher mortality in women with Turner syndrome (TS). It has also been suggested that the occurrence of these diseases is dependent on karyotype., Aim: To assess the cardiovascular system by clinical examination, echocardiography and electrocardiography in female patients with TS, depending on karyotype., Patients and Methods: Thirty-four women with TS, aged 7-58 (35.2 +/-14.6) years, were evaluated. The patients were categorized into 3 groups according to karyotype: group 1 comprised 12 patients with monosomy X (45,X), group 2 comprised 19 women with mosaicism, and group 3 comprised 3 patients with structural aberrations of X chromosome. Echocardiography was performed using the Sonos 5500 machine, following the current standards., Results: Congenital cardiovascular malformations were detected in 21% of TS women. Valvular heart disease was present in 50% of the entire study population, including aortic valve diseases in 24% and mitral valve diseases in 21%. The patients with mosaicism, more often than those with monosomy X, were overweight and had a tendency to higher incidence of hypertension, as well as thicker interventricular septum, and larger left ventricular mass on echocardiography., Conclusions: Congenital cardiovascular malformations are more common in TS patients compared to the general population. There were no differences in risk factors and cardiovascular abnormalities between various karyotypes except for higher incidence of overweight and tendency to higher blood pressure, thicker interventricular septum, and larger left ventricular mass observed in patients with mosaicism compared to those with monosomy X.

Background: Dieulafoy lesion is a rare but serious cause of gastrointestinal system bleeding. An aberrant submucosal artery, which was described in 1884, causes the bleeding. The lesion can be located anywhere in the gastrointestinal tract but is most commonly found in the proximal stomach up to 6 cm from the gastroesophageal junction. Increased experience in endoscopy has led to an increased frequency of its proper diagnosis. Various methods are used to achieve successful hemostasis by endoscopy in Dieulafoy lesion; however, comparative studies about the success rates of these methods are still needed. In this study, we compared two of these endoscopic hemostatic methods: band ligation, and injection therapy in Dieulafoy lesions., Methods: In this prospective study, 18 patients admitted to the Emergency Surgical Unit between January 2002 and December 2005 with upper gastrointestinal bleeding diagnosed as Dieulafoy lesion were included. Diagnose of Dieulafoy lesion was made at initial or second-look endoscopy. Patients were randomized in two groups according to therapy method: injection therapy and band ligation groups. Therapy was applied immediately after recognizing the lesion at the same endoscopic procedure. Two groups were compared regarding demographical data, presence of comorbid diseases, history of medication and previous gastrointestinal system bleeding, hemodynamic status, laboratory values, need for transfusion, endoscopic findings, success rate of the treatment method, mean hospital stay, complications, and recurrence of bleeding., Results: Of 588 patients admitted with upper gastrointestinal hemorrhage, Dieulafoy lesion was recognized in 18 cases (3.1%) at initial or second-look endoscopy. All patients were men with a mean age of 62.8 (range, 30-80) years. Band ligation was applied to ten patients and the remaining eight were treated by injection therapy. During the follow-up period, rebleeding occurred in six of the patients (75%) with injection therapy, whereas no rebleeding occurred for the patients in the band ligation group. The rebleeding rate and mean hospital stay was significantly higher for the injection therapy group., Conclusions: Our study suggests that of the endoscopic treatment methods, band ligation is superior to injection therapy for the treatment Dieulafoy lesions.

Studies of larger patient groups for systematic assessment of the anatomical accuracy of magnetic resonance imaging (MRI) for partial anomalous pulmonary venous drainage (PAPVD) have been performed so far only in adults. This study was undertaken to evaluate whether MRI can precisely depict pulmonary venous anatomy in infants and young children. Data on 26 children under 10 years old that underwent MRI over the past 2 years for suspected PAPVD were assessed. The MRI protocol included shunt quantification by velocity-encoded cine as well as morphological and functional assessment by multislice multiphase and contrast-enhanced MR techniques. MRI was performed in the compliant patient in breath-hold (n = 8; age range, 4.6-9.5 years) and in the noncompliant patient in conscious-sedation free breathing (n = 18; age range, 0.4 to 7.5 years). In 22 patients, PAPVD was diagnosed with MRI and confirmed during surgery. In four patients with large atrial septal defects not accessible to percutaneous closure, normal pulmonary venous return was demonstrated by MRI and confirmed during surgery. MRI under conscious sedation accurately specifies the anatomy of pulmonary veins in infants and small children. Therefore, we suggest performing MRI in patients with inconclusive transthoracic echocardiographic results in the preoperative assessment of PAPVD.

The accidental discovery of a retrotracheal left pulmonary artery in a 4-month-old infant encouraged us to review the various embryologic theories concerning this very rare anomaly and perform an anatomic update in order to better define surgical treatment. Nathan underwent surgery for a bilateral inguinal hernia at the age of 4 months. The postoperative period was marked by malaise associated with dyspnoea, stridor, tachycardia and sweating. A X-ray of the thorax, oesophageal transit and angio scan presented an intertracheo-oesophageal left pulmonary artery and a reimplantation of the left pulmonary artery was successfully performed. A retrotracheal left pulmonary artery is a very rare malformation. From development of pulmonary vascularisation, three embryologic theories have been advanced to explain this anomaly. From an anatomic point of view, Landing et al. proposed in 1982 a classification system of retrotracheal left pulmonary artery. Today, current radiological techniques not only provide a precise diagnosis but also make it possible to define appropriate care for the different types of this malformation.

Large, segmental, cervicofacial hemangiomas are often associated with anomalies of intracranial, cardiovascular, and ophthalmic systems as well as ventral development.

Objectives: To describe the otolaryngic manifestations of PHACES and evaluate current diagnostic and management principles for these patients., Methods: A retrospective review was performed within a tertiary children's hospital. Children with segmental facial hemangiomas of infancy and one extracutaneous manifestation comprising PHACES (posterior fossa malformation, arteriovenous malformations, cardiac/aortic defects, eye anomalies, and sternal defect) were identified. Otolaryngic problems were evaluated with physical examination, audiogram, swallow evaluation, polysomnography, and laryngoscopy. Extracutaneous manifestations were diagnosed using radiology, echocardiogram, and EEG. Treatment for cutaneous and airway hemangiomas included oral and intralesional steroids, CO(2) or pulse-dye laser, tracheotomy, and surgical excision. Management of extracutaneous problems was system-dependent., Results: Of 246 children with segmental facial hemangiomas of infancy evaluated since January 2000, 5 girls (2.0%) met diagnostic criteria for PHACES. Mean age at last follow-up was 2.6 years (range 0.4-5.8). Each child had one extracutaneous manifestation of aortic anomaly (2/5), sternal clefting (2/5), and brain malformation (1/5). Otolaryngic abnormalities included middle ear atelectasis (1/5), tympanic membrane hemangiomas with conductive hearing loss (3/5), skin and cartilage ulceration (2/5), dysphagia (4/5), and airway hemangiomas with stridor (3/5). Three children received oral steroids and required pulse-dye laser for cutaneous hemangiomas of infancy. One child underwent tracheotomy. Additional interventions included tympanostomy tubes and resection of nasal hemangioma., Conclusions: Diagnosis of PHACES requires awareness of the association of facial hemangiomas of infancy with systemic and airway problems. Otolaryngology-related manifestations of PHACES are not commonly described, and management should be tailored to the individual patient.

Persistent left superior vena cava is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress. It is most commonly observed in isolation but can be associated with other cardiovascular abnormalities including atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, and cor triatriatum. The presence of PLSVC can render access to the right side of heart challenging via the left subclavian approach, which is a common site of access utilized when placing pacemakers and Swan-Ganz catheters. Incidental notation of a dilated coronary sinus on echocardiography should raise the suspicion of PLSVC. The diagnosis should be confirmed by saline contrast echocardiography.

Background: Asplenia or polysplenia and complex cardiovascular defects, in association with disturbed body symmetry and malposition of internal organs, constitute the main corpus of malformations in the heterogeneous group of heterotaxy disorders. In affected pregnancies, prenatal diagnosis is possible by ultrasonography, while prognosis and counseling largely depend upon the severity of the cardiac defect., Case: We present a 25 week gestation fetus with typical findings of asplenia-cardiovascular defect-heterotaxy (Ivemark syndrome) and aplasia of the pancreas., Conclusions: Pancreatic aplasia emerges as an additional phenotypic feature in Ivemark syndrome and raises the possibility of total pancreatic insufficiency of the affected neonate as an additional, although rare, clinical consideration., (Copyright 2008 Wiley-Liss, Inc.)

The case of a young male patient with a complex admixture lesion who required a comprehensive anatomic evaluation before palliative cardiac surgery is presented. We describe a safe anesthesia protocol for obtaining the late-acquisition, gadolinium-enhanced, magnetic resonance angiographic images necessary to define the complex pulmonary and systemic venous anatomic features of his cardiac admixture lesion. Subspecialty physician staffing implications for the care of military dependants with congenital heart disease who might benefit from evaluation using this safe simple protocol and readily available magnetic resonance imaging technology are addressed.

Carotid-femoral pulse wave velocity (cf-PWV) is a validated marker of arterial stiffening over the central arteries. Brachial-ankle pulse wave velocity (ba-PWV) integrates the mechanical properties from both the central and peripheral arteries and may be more representative than cf-PWV as arterial load for left ventricle (LV). We compared ba-PWV with cf-PWV for the association of cardiovascular structure and function in 320 subjects with various degrees of abnormality in cardiac structure and function. ba-PWV (by oscillometric technique) and cf-PWV (by tonometric technique) were measured simultaneously, and were highly correlated (r=0.79, P<0.001). Both ba-PWV and cf-PWV were significantly correlated with LV mass, but the correlation was better with ba-PWV (r=0.29 vs r=0.22, P=0.0219). While ba-PWV and cf-PWV were similarly significantly correlated with LV end-systolic elastance and mitral E/A ratio, ba-PWV had better correlation with isovolumic relaxation constant (r=0.34 vs r=0.27, P=0.0202) than cf-PWV. In addition, the correlation was also significantly stronger with ba-PWV than with cf-PWV for other indices of arterial stiffness, including carotid incremental modulus (r=0.59 vs 0.50, P=0.0013), effective arterial elastance (r=0.41 vs r=0.33, P=0.0081) and carotid augmentation index (r=0.38 vs r=0.32, P=0.0368). In conclusion, ba-PWV correlates better with LV mass and diastolic function and other indices of arterial function than cf-PWV, probably because ba-PWV encompasses a greater territory of arterial tree than cf-PWV.

Congenital diaphragmatic hernia (CDH) is a common birth defect that can be subdivided into several different types depending on the anatomical structures affected. Several studies have demonstrated that right-sided CDH (R-CDH) and left-sided CDH (L-CDH) in humans are associated with different clinical presentations and outcomes. We hypothesized that R-CDH and L-CDH are phenotypically and pathogenically distinct, and that the frequency of associated anomalies would differ among patients with R-CDH compared to patients with L-CDH. Using population-based data from ongoing studies in the California Birth Defects Monitoring Program (CBDMP), we compared 146 CDH cases, 38 with R-CDH (26%) and 108 with L-CDH (74%). The frequencies of atrial septal defect (1/38 R-CDH and 20/108 L-CDH cases; P = 0.015), bilateral pulmonary hypoplasia (22/108 L-CDH cases and 15/38 R-CDH cases; P = 0.029), abnormal skull or facial shape (17/108 L-CDH patients and 1/38 R-CDH cases; P = 0.043), assorted digital anomalies excluding syndactyly, polydactyly or absence of a digit (13/108 L-CDH patients and 0/38 R-CDH patients; P = 0.021) and assorted limb anomalies excluding limb reduction defects (18/108 L-CDH patients and 0.38 R-CDH patients; P = 0.004) all showed a statistically significant difference in frequency between R- and L-CDH patients. This descriptive epidemiological study using newborn surveillance data represents the largest comparison of right and left-sided CDH patients so far performed. Several anomalies as listed above were noted to be different in frequency between R- and L-CDH patients, although the interpretation of the significant variability in the frequency of these anomalies is not yet established and the findings require replication. Birth defects monitoring programs can provide the interface for epidemiology and clinical genetics, which in this case, may generate hypotheses to establish the pathogenesis of CDH laterality. (c) 2007 Wiley-Liss, Inc., ((c) 2007 Wiley-Liss, Inc.)

Acardiac twinning is a rare anomaly .75% of cases occurring in monzygotic triplet pregnancies and the rest in monozygotic twins. We report an antenatally undiagnosed case of acardius amorphous. The condition results from abnormal placental vascular anastomoses termed as twin reversed arterial perfusion (TRAP) theory. Early sonographic prenatal diagnosis improves the survival of the normal twin called the pump twin.

Objective: To evaluate the cardiovascular manifestations of Williams syndrome (WS) confirmed by fluorescence in situ hybridization (FISH)., Methods: Between July 2004 and January 2007, FISH was used to confirm diagnosis in 71 suspected WS cases by detecting chromosome 7q microdeletion. Cardiovascular abnormalities were assessed by echocardiography and Doppler echocardiography., Results: Forty out of 71 patients were detected to have Elastin gene locus microdeletion, 25 patients (25/40, 62.5%) had at least one cardiac anomaly; among these patients, supravalvular aortic stenosis (SVAS) was diagnosed in 18 patients (18/25, 72%) and 6 of them had complex abnormalities. Patent ductus arteriosus was diagnosed in 3 patients (3/25, 12%, 1 was associated with other malformations), isolated pulmonary stenosis in 1 patient (1/25, 4%), isolated coarctation of aorta in 2 patients (2/25, 8%), and hypertension in 2 patients (2/25, 8%), mild aortic regurgitation in 2 patients, mild mitral regurgitation and moderate mitral regurgitation in 3 patients respectively., Conclusion: A detailed cardiac evaluation should be performed in all patients with Williams syndrome due to the high frequency of cardiovascular abnormalities.

During the implantation of a definitive pacemaker for complete atrioventricular block via the left subclavian vein, a rare and abnormal origin of the superior vena cava was detected. No such finding has ever been reported, leading us to adopt the contralateral approach.

Isolation of the left subclavian artery is a rare aortic arch anomaly in which the artery originates from the pulmonary artery through a ductus arteriosus rather than the aorta. In neonates it is usually diagnosed incidentally with other aortic or cardiac anomalies and can be associated with chromosomal deletions. We describe an extremely rare presentation whereby subclavian artery isolation was presented with left arm ischemia in a 6-day-old child. There were also a concurrent right aortic arch, ventricular septal defect, persistent left superior vena cava, and both radial and ulnar artery hypoplasia. A family history of aortic arch anomalies with no known chromosomal aberration was also present, whereby all the male family members on the maternal side had undergone aorto-cardiac surgery as neonates. Diagnosis and surgical management are discussed.

Pulmonary vein pathologies often present a diagnostic challenge. Among the different imaging modalities used for the evaluation of pulmonary veins, magnetic resonance is the most comprehensive in assessing anatomy and pathophysiology at the same time. Bright blood cine sequences and contrast-enhanced magnetic resonance angiography outline the course and connections of the pulmonary veins. Phase-contrast velocity mapping measures flow patterns, velocities, and volumes throughout the pulmonary circulation. This paper reviews contemporary utilization of magnetic resonance in the evaluation of pulmonary venous abnormalities in children, based on our experience over the last 5 years and on that of other investigators. We summarize how magnetic resonance imaging enhances our understanding of pulmonary vein physiology and how it can influence the diagnostic approach to children and adults with a pulmonary venous pathology, and we discuss its limitations.

Although uncommon and usually incidental findings on imaging or at operation, congenital anomalies of the retroperitoneal venous system, particularly those involving the inferior vena cava and left renal vein, can be troublesome and dangerous during open repair of abdominal aortic aneurysms (AAA). We have provided a retrospective analysis of our single tertiary institution experience with these anomalies during more than 2400 open AAA reconstructions in an effort to determine if preoperative identification of these vessels decreased venous injury and subsequent morbidity as well as offering a thorough review of the relevant embryology, anatomy, and technical approaches for each specified anomaly for the practicing vascular surgeon.

In women with Turner syndrome, the risk for aortic dissection or rupture during pregnancy may be 2% or higher, and the risk of death during pregnancy is increased as much as 100-fold.

A case of an actively bleeding mediastinal mass in a 4-year-old boy who sustained multitrauma is described. A computed tomography (CT) scan of the chest upon admission demonstrated a mediastinal mass, which enlarged significantly as seen by repeat CT scan at 3 days with a concomitant drop in serum hemoglobin levels. The lesion was excised, and pathological examination established the diagnosis of a lymphatic-venous malformation containing bloody fluid. Lymphatic-venous malformations are rare vascular malformations and are usually found in the head and neck and less commonly at other sites. Spontaneous bleeding is a known complication, and there are few reports describing posttraumatic hemorrhage. Our case is the first description in the English medical literature of a posttraumatic bleeding mediastinal mixed vascular malformation and includes sequential CT scans illustrating the dynamic nature of hemorrhage.

Purpose: The prevalence of most minor cardiovascular manifestations in Marfan syndrome (MFS) is unknown. We assessed the prevalence of minor cardiovascular manifestations in MFS to evaluate their usefulness in a diagnostic setting., Methods: Seventy-seven patients with MFS (aged 4 months to 55 years) underwent echocardiography to assess the presence of mitral valve prolapse and the diameter of the main pulmonary artery. A subset of 29 adult patients with MFS also underwent magnetic resonance imaging evaluation of the diameters of the thoracoabdominal aorta., Results: Mitral valve prolapse was encountered in 66% of patients with MFS, with an equal distribution of classic and nonclassic mitral valve prolapse. The main pulmonary artery diameter was significantly larger in patients with MFS at all ages when compared with controls. In the adult group (> or = 14 years), we were able to provide a cutoff value of 23 mm to define pulmonary artery dilatation. The descending aorta was enlarged, but with substantial overlap with controls, thus precluding the use of a cutoff value., Conclusions: Mitral valve prolapse and main pulmonary artery dilatation are common findings in MFS patients at all ages and are easy to assess with echocardiography. Cutoff values to define dilatation of the descending aorta are hard to define, making them of limited value in the diagnostic evaluation. We recommend echocardiographic evaluation of mitral valve prolapse and main pulmonary artery diameter in patients referred for cardiovascular diagnostic assessment for MFS.

The objective of this study was to determine if distortion of the left subclavian artery course distinguishes double aortic arch with atretic left dorsal aorta from right aortic arch with mirror image branching. We performed a retrospective case series at a tertiary care center. Twenty-six patients undergoing magnetic resonance imaging for suspicion of a vascular ring were identified, 6 of whom had an atretic left dorsal aorta confirmed by surgical inspection. Six patients with the diagnosis of right aortic arch with mirror image branching were identified for comparison. The course of the left subclavian artery was assessed using surface-rendered magnetic resonance angiography (MRA) and axial fast spin echo images. All patients with double aortic arch had clinical symptoms suggestive of esophageal or tracheal compression. Six patients had double aortic arch, 3 of whom had an atretic left dorsal aorta. In these 3 patients, the branching patterns on MRA mimicked right aortic arch mirror image branching except for the distortion of the initial course of the left subclavian artery. Surgical observation confirmed the presence of an atretic left dorsal aorta that resulted in tension on the left subclavian artery pulling it posteriorly and inferiorly and completing the vascular ring. Patients with right aortic arch mirror image branching demonstrated no such subclavian artery distortion, and these patients did not have clinical symptoms suggestive of a vascular ring. Our results demonstrate that left subclavian artery distortion due to traction by an atretic left arch is an important diagnostic finding in the evaluation 6 patients with suspected vascular rings.

Unlabelled: We aimed to assess the prevalence of ECG abnormalities in children with idiopathic scoliosis (IS). 77 Girls and 13 boys, aged 7 to 18 years (15 +/- 3), including 12 with thoracic scoliosis (mean Cobbe angle 33 degrees), 4 with lumbar scoliosis (29 degrees), 12 with thoracolumbar (27 degrees), and 62 with double-major scoliosis (31 degrees) entered the study. They were grouped as follows: 20 children <14 years of age (A), 35 from 14 to 16 (B), and 35 >16 years of age (C). Routine ECG was recorded and analysed automatically (GE, CASE v.4.1). Several ECG indices were further analysed. Abnormal values were considered if they exceeded upper normal limit (>95 percentile)., Results: Abnormalities were found in 66 patients (73%) independent of age. QRS duration gt;90 ms was observed in 40 patients (44%), right axis deviation in 28 (31%), left axis deviation in 2 (2%). The Rsr'(V1-2 ) pattern was noticed in 25 patients (28%). Ventricular gradient gt;60 degrees was found in 11 patients (12%). In patients with normal ECG there was a normal leftward axis rotation with age (-10 degrees +/-18 degrees in gr.A, -5 degrees +/-17 degrees in gr.B and +2 degrees +/-22 degrees in gr.C, difference from median), whereas reversed trend (rightward deviation with age) characterized patients with ECG abnormalities (-7 degrees +/-30 degrees in gr.A, +9 degrees +/-19 degrees in gr.B and +19 degrees +/-2 degrees in gr.C, p<0.001)., Conclusion: In children with idiopathic scoliosis, subtle ECG abnormalities are frequent. Abnormal trend of rightward QRS axis deviation with age suggests cardiac involvement in natural history of scoliosis and requires more depth cardiac evaluation.

Thoracic impedance cardiography is a noninvasive hemodynamic monitoring technique based on the physical principal of impedance, which can be defined as resistance to electrical flow. We report the case of a man who underwent orthotopic heart transplantation, during which the superior vena cava was found to drain into the coronary sinus. Impedance cardiography was used because hemodynamics could not be monitored invasively with a Swan-Ganz catheter. We describe the main applications of impedance cardiography and the characteristics of this rare vascular anomaly.

Patients with congenital central hypoventilation syndrome (CCHS) (Ondine's curse syndrome) have impaired autonomic control of ventilation with intact voluntary control of respiration. Autonomic dysfunction and cardiac abnormalities are common in CCHS. Bradyarrhythmias are life-threatening and often require pacemaker insertion. We presented a case of a patient with CCHS suffering from long sinus pauses requiring cardiac pacemaker insertion. Patients with CCHS are at risk for pulmonary hypertension and cor pulmonale secondary to chronic hypoxia. Diaphragmatic pacing has been beneficial in some patients with CCHS. In this article, we review concomitant cardiac abnormalities and the occurrence of bradyarrhythmias in patients with CCHS.

Primary evaluation of congenital cardiac abnormalities traditionally relies upon echocardiography and conventional angiography, both of which have potential limitations. Echocardiography is an operator dependant study, limited by a small window and patient movement. Conventional angiography is an invasive procedure with an inherent risk of catheter complication such as vessel damage, bleeding, stroke and infection. During angiography, overlapping of the pulmonary and systemic circulation often provides a confusing picture given complex anatomy. Another limiting factor of particular significance in young children is radiation dose and contrast administration during catherization procedures. Three-dimensional MDCT provides an alternative to alleviate these pitfalls of traditional cardiac diagnostic studies. Development of multi-detector computed tomography (MDCT) and 3D software provides new methods for non-invasive visualization and evaluation of congenital cardiac abnormalities. The multiplanar, volumetric functions allow faster and more complete computed tomography diagnosis and better understanding of clinical relevance of complex cardiac anatomy. In addition, 3D imaging is particularly useful for preoperative planning and postoperative outcomes. This essay provides case studies to illustrate the usefulness of MDCT/3D CT for evaluation of complex congenital heart disease.

Holoprosencephaly is one of the rare types of craniofacial congenital anomaly characterized by failure of differentiation of the procencephalon to diencephalon and telencephalon derivatives that accompanied by variable degree of orbitofacial dysmorphism. The case presented to our hospital in the third trimester of pregnancy with intrauterine fetal death was diagnosed prior to delivery as hydrocephalus to rule out hydrops fetalis. Autopsy examination showed distinctive features of holoprosencephaly of severe type in conjunction with a large head fused with the thorax to appear as a spherical ball ('cephalothorax' nature) and multiple cardiac malformations but with normally differentiated upper and lower limbs, intestines, female reproductive system and kidneys. We are reporting this unusual case to remind clinicians to be conscious on partly preventable teratogens specific to this anomaly, and if failed to do so, to diagnose as early as possible and terminate the pregnancy through vaginal route, within all the provision of the law.