1/22. Complicated persistent patent ductus arteriosus with acute pneumonia in an adult.Patent ductus arteriosus (PDA) is a malady usually identified during childhood. Prompt surgical correction provides definitive therapy with long-term survival. When not corrected, PDA leads to significant morbidity and mortality, making it a rare condition in the adult population. We report the case of a 44-year-old man with a history of persistent PDA admitted for worsening dyspnea and fever. Radiographic studies are illustrative of this complex syndrome. We review the complications and treatment alternatives in these patients.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/22. Unusual form of coarctation of the distal thoracic aorta.We report the case of a 3-month-old girl with a rare form of coarctation involving the lower descending thoracic aorta. Because of clinical findings of congestive heart failure and hypertension, early repair was recommended. Surgical intervention in young patients with this unusual localization presents a complex challenge. Aortic reconstruction was carried out by patching the stenotic segment with autologous arterial tissue. Three years after the repair, there is no evidence of recoarctation or aneurysmal dilation.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
3/22. Interruption of the aortic arch associated with patent ductus arteriosus and ventricular septal defect. Proposal of a new surgical technique for total correction.A four-year-old girl underwent complete correction of the association of interruption of the aortic arch (Type A, Celoria and Patton's classification), patent ductus arteriosus, and ventricular septal defect. The surgical procedure consists of establishment of a continuity between the ascending and descending aortas utilizing the patent ductus arteriosus and the anterior wall of the pulmonary arterial trunk, reconstruction of the rest of pulmonary arterial trunk with pericardium, and closure of the ventricular septal defect. Though the patient expired from cerebral complications, the hemodynamic result after repair was quite satisfactory. The procedure described makes total correction of this complex anomaly feasible at one operation through a median sternotomy and seems to be a method of choice for most patients with this association of anomalies, unless there is severe narrowing of the patent ductus arteriosus.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
4/22. An N-terminal WT1 mutation (P181S) in an XY patient with ambiguous genitalia, normal testosterone production, absence of kidney disease and associated heart defect: enlarging the phenotypic spectrum of WT1 defects.OBJECTIVE: This study reports the clinical and molecular data of an XY patient with a very unusual phenotype due to a Wilms' tumor-suppressor (WT1) gene mutation. The genotype-phenotype relationship of different WT1 mutations is then discussed. PATIENT: The patient presented at birth with micropenis, severe hypospadias and cryptorchidism. Normal androgen production and an absence of clinical response to a testosterone treatment trial suggested partial androgen resistance. Eventually, female sex of rearing was chosen. At the beginning of puberty, normal male androgen production occurred, and subsequent gonadectomy did not show gonadal dysgenesis. It is notable that the patient, now 20 years of age, has not developed kidney disease. In addition to the genital malformation, the patient displayed an associated congenital heart defect, consisting of a coarctation of the aorta and a patent ductus arteriosis (PDA). RESULTS: No mutations were detected in the androgen receptor or 5alpha-reductase genes. Direct sequencing of the WT1 gene identified a heterozygous proline to serine substitution at position 181 (P181S). The same heterozygous mutation was found in the mother. Interestingly, the mother shows no signs of kidney disease at her present age of 49. CONCLUSION: This is the first germline missense mutation in the N-terminal part of WT1 identified in a patient with the very particular phenotype of ambiguous genitalia with absence of gonadal dysgenesis and kidney disease. The possible molecular mechanisms leading to the patient's phenotype are considered. The high frequency of PDA in newborns and the absence of heart abnormalities in XX females carrying the P181S mutation, however, suggest that the heart defect was most likely a coincidental association. This case enlarges the clinical spectrum of WT1 defects and may provide new insights into the complex functions of WT1 in genital and kidney development.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
5/22. heart-lung transplantation in korea.heart-lung transplantation is an effective treatment for patients with various forms of congenital heart disease or pulmonary hypertension. Since the first heart-lung transplantation in 1997, five transplants have been performed in korea. Three cases were performed in 1997, one in 1998, and the latest one in 2002. The preoperative diagnoses were complex congenital heart disease (CHD) in 2, and CHD with Eisenmenger's syndrome in 3. In this paper, we report five cases of heart-lung transplantation performed in korea, and include a review of the relevant literature.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
6/22. Anesthetic management of conjoined twins presenting for palliative open-heart surgery.A set of dicephalous parapagus twins was born at 36 wk gestational age. Twin A had complex congenital heart disease in the form of a single ventricle that would be fatal without surgical intervention. Twin B had normal intracardiac anatomy. The twins were deemed surgically inseparable and the parents requested palliative open-heart surgery on Twin A. The twins shared a common circulatory system to the lower half of the body so that physiological manipulations in one twin adversely affected the other's hemodynamic condition. Twin A underwent successful single ventricle palliation; however, after a prolonged hospitalization, the infants died secondary to severe respiratory disease in Twin B.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
7/22. Left hemitruncus in adulthood: diagnostic role of magnetic resonance imaging.The diagnosis of left hemitruncus and large patent ductus arteriosus was made by magnetic resonance imaging in an adult patient with recurrent haemoptysis and dyspnoea on exertion. Previous cardiac catheterization and echocardiography failed to establish the complete diagnosis. magnetic resonance imaging using spin-echo and gradient-echo pulse sequences is a useful imaging modality to evaluate anatomical and functional abnormalities in patients with complex congenital heart disease.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
8/22. Anaesthetic management of congenital heart block and persistent ductus arteriosus in an infant.ketamine hydrochloride, pancuronium bromide and 100% oxygen were used during general anaesthesia for a left antero-lateral thoracotomy, insertion of a permanent cardiac pace-maker and ligation of a persistent ductus arteriosus in a three-month-old female infant, who was also in congestive cardiac failure. To the best of our knowledge, this is the first reported case of successful anaesthetic management for insertion of cardiac pace-maker and ligation of persistent ductus arteriosus under general anaesthesia in a Nigerian infant. The successful out-come of our anaesthetic technique encourages us to recommend the procedure for the anaesthetic management of similar complex cardiac abnormalities.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
9/22. Transcatheter occlusion of ventricular septal defect.The patent ductus arteriosus occlusion device (Rashkind-USCI) was employed to occlude a residual ventricular septal defect after fontan procedure in a patient with hypoplastic right ventricle, ventricular septal defect, and pulmonary stenosis. There was significant right-to-left shunting across the ventricular septal defect, with cyanosis exacerbated by exercise. After placement of the 17 mm occlusion device, the right-to-left shunt was markedly diminished, and the cyanosis resolved. Occlusion of ventricular septal defects in patients with complex congenital heart disease may be performed with the patent ductus arteriosus occluder in selected instances.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
10/22. Exogenous prostaglandin administration and pseudo-bartter syndrome.Biological abnormalities simulating bartter syndrome were observed in a preterm neonate with complex cyanotic congenital heart disease, for which ductus arteriosus was maintained open by high doses of prostaglandin (PG) until a Blalock shunt could be performed. These abnormalities spontaneously disappeared after cessation of PG administration. We postulate that the natriuretic effect of exogenous administered PG could further increase sodium wasting already induced by the cardiopathy thus leading to pseudo-bartter syndrome.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
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