Outcome of pregnancy in women with congenital shunt lesions.
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OBJECTIVE: To evaluate the outcome of pregnancy in women with congenital shunt lesions. SETTING: Retrospective study in a tertiary care centre. METHODS: Pregnancy history was obtained by a standardised questionnaire and medical records were reviewed. PATIENTS: 175 women were identified, at a mean (SD) age of 42 (14) years. Pregnancies occurred in 126 women: 50 with an atrial septal defect, 22 with a ventricular septal defect, 22 with an atrioventricular septal defect, 19 with tetralogy of Fallot, and 13 with other complex shunt lesions. RESULTS: 309 pregnancies were reported by 126 woman (2.5 (1.6) pregnancies per woman). The shortening fraction of the systemic ventricle was 40 (8)%, and 98% were in New York Heart Association class I-II at last follow up. Spontaneous abortions occurred in 17% of pregnancies (abortion rate, 0.4 (0.9) per woman). Gestational age of the 241 newborn infants was 8.8 (0.8) months. There were no maternal deaths related to pregnancy. Pre-eclampsia and embolic events were observed in 1.3% and 0.6%, respectively of all pregnancies. Women with complex shunt lesions more often underwent caesarean section (70% v 15-30%, p = 0.005) and gave birth to smaller babies for equivalent gestation (2577 (671) g v 3016 (572) to 3207 (610) g, p < 0.05). The recurrence risk of congenital heart disease was 2.5%. CONCLUSIONS: The outcome of pregnancy is favourable in women with congenital shunt lesions if their functional class and their systolic ventricular function are good. Such patients can be reassured. (+info)
Mechanisms of deficient cardiac septation in the mouse with trisomy 16.
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It used to be thought that the atrioventricular septum was predominantly the product of the atrioventricular endocardial cushions. In a previous study, we have shown that multiple developmental primordia are of importance in its formation. With this in mind, we have evaluated cardiac morphogenesis in the mouse with trisomy 16, an animal model with a high incidence of atrioventricular septal defects. Normal and trisomic fetuses from an Rb(11.16)2H/Rb(16.17)7Bnr x C57BL/6J cross were collected on days 10 to 15 of gestation and examined by scanning electron microscopy and histological serial sectioning. No evidence was found to suggest that atrioventricular septal defect could be explained simply on the basis of "failure of fusion" between the atrioventricular endocardial cushions. Rather, our findings supported two other developmental elements as being important in the genesis of atrioventricular septal defect. The first is an alteration in the configuration of the heart tube, with inadequate remodeling of the inner heart curvature. This resulted in the failure of the atrioventricular junction to expand to the right, with subsequent malalignment of the atrioventricular endocardial cushions with the proximal outflow cushions. The second is a variability in the connection of the primary atrial cardiac segment to the body of the embryo, the so-called dorsal mesocardium, which influences its relationship to the extracardiac mediastinal mesoderm. There appeared little difference in the connection between normal and trisomic embryos at the stage of 20 to 25 somites, but the area subsequently showed marked changes. In most trisomic embryos, the connection with the mediastinal mesoderm of the body was over a larger area than seen in normal embryos. As this area of attachment encloses the pulmonary pit, the entry point of the pulmonary vein, this gives potential for variation in the connection of the pulmonary vein. In addition, in the majority of trisomic embryos, the right pulmonary ridge (the spina vestibuli) did not accumulate extracardiac mesoderm, nor did it undergo the pronounced forward growth seen in normal embryos of equivalent stages. Consequently, the trisomic embryos show incomplete formation of both the atrial and the atrioventricular septal structures. (+info)
Methodological parameters influence the detection of right-to-left shunts by contrast transcranial Doppler ultrasonography.
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BACKGROUND AND PURPOSE: Contrast transcranial Doppler ultrasonography is a new method to detect intracardiac right-to-left shunts, such as the patent foramen ovale. However, the methodology of the procedure varies considerably among investigators. This study was undertaken to assess the influence of methodological parameters on the results of the contrast transcranial Doppler examination in the detection of right-to-left shunts. METHODS: A total of 72 patients (mean age, 58.2+/-14.7 years) had a contrast transcranial Doppler ultrasonography examination. To study the influence of methodological factors, patients with evidence of a right-to-left shunt underwent repeated examinations with modified procedures. Parameters under investigation were the timing of the Valsalva maneuver, the dose of the contrast medium, and the patient's posture during the examination. RESULTS: The median contrast signal count was 58.5 and 48.0 (P<0.001) and the median latency of the first intracranially detected contrast signal was 12.5 and 8.5 seconds (P=0.05) when the Valsalva maneuver was performed 5 and 0 seconds after the start of the injection, respectively. Reducing the contrast medium dose from 10 to 5, 2.5, and 1.2 mL resulted in a decline of the median signal count from 54.5 to 28.5, 20.5, and 12.0 (P<0.01), respectively, while the latency of the first contrast signal increased from 13.3 to 14.0, 14.6, and 15.0 seconds (P<0.05). The sitting position also produced a lower signal count than the supine position (P<0.02). CONCLUSIONS: This study demonstrates that several essential methodological parameters influence the results of the contrast transcranial Doppler ultrasonography examination. Therefore, it is necessary to standardize the procedure to permit comparable quantitative assessments of the shunt volume. The findings of the present study suggest that 10 mL of contrast medium be injected with the patient in the supine position and that the Valsalva maneuver be performed 5 seconds after the start of the injection. (+info)
Effect of nebulized epoprostenol (prostacyclin) on exhaled nitric oxide in patients with pulmonary hypertension due to congenital heart disease and in normal controls.
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Inhaled epoprostenol (prostacyclin) may be used in the treatment of severe pulmonary hypertension, improving oxygenation and reducing pulmonary artery pressures. We have observed symptomatic benefits of epoprostenol in patients with congenital heart disease that extend beyond acute haemodynamic effects of the drug, which has a short biological half-life. The aim of this study was to examine the effects of epoprostenol in patients and normal subjects on exhaled nitric oxide (eNO), based on the hypothesis that the drug may alter the resting vasoconstrictor/vasodilator balance. Nine patients with pulmonary hypertension complicating left-to-right cardiac shunts and nine healthy controls received 100 microgram of nebulized epoprostenol. Exhaled eNO was measured, using a chemiluminescence method, before, immediately after and 18 h after nebulization. There was no significant difference between the two groups in baseline eNO or eNO immediately following nebulized epoprostenol. Epoprostenol produced a delayed elevation in eNO 18 h after nebulization in patients, but not in normal controls. This study supports the concept that epoprostenol, while having no effect on the normal pulmonary circulation, acts on the hypertensive circulation via a mechanism that may result in a delayed alteration of vasoconstrictor/vasodilator balance. (+info)
Localization of the human caveolin-3 gene to the D3S18/D3S4163/D3S4539 locus (3p25), in close proximity to the human oxytocin receptor gene. Identification of the caveolin-3 gene as a candidate for deletion in 3p-syndrome.
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Caveolin-3, a muscle-specific caveolin-related protein, is the principal structural protein of caveolae membrane domains in striated muscle cell types (cardiac and skeletal). Recently, we identified an autosomal dominant form of limb girdle muscular dystrophy in humans that is due to mutations within exon 2 of the caveolin-3 gene (3p25). However, the detailed location of the human caveolin-3 gene and its position with regard to neighboring genes remains unknown. Here, we have isolated three independent BAC clones containing the human caveolin-3 gene. Using a PCR-based approach, we determined that these clones contain both exons 1 and 2 of the human caveolin-3 gene. In addition, we performed microsatellite marker analysis of these BAC clones, using a panel of 13 markers that are known to map within the 3p25 region. Our results indicate that these BAC clones contain the following three markers: D3S18, SHGC-1079 (also known as D3S4163) and D3S4539. Interestingly, D3S18 is a marker for two known human diseases, von Hippel-Lindau disease and 3p-syndrome. As D3S4163 and D3S4539 are known to map in the vicinity of the 3' end of the human oxytocin receptor gene, we determined if these caveolin-3 positive BACs also contain the oxytocin receptor gene. We show that (i) these BACs contain all four exons of the oxytocin receptor gene and (ii) that the genes encoding caveolin-3 and the oxytocin receptor are located approximately 7-10 kb apart and in the opposite orientation. As 3p-syndrome is characterized by cardiac septal defects and caveolin-3 is expressed primarily in the heart and skeletal muscle, caveolin-3 is a candidate gene that may be deleted in 3p-syndrome. (+info)
Open-heart surgery in 48 patients via a small right anterolateral thoracotomy.
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To limit the trauma to the chest and to achieve a pleasing cosmetic result, we used 2 types of right anterolateral thoracotomy in 48 patients who required open-heart surgery: 1 was a curved incision along the lower edge of the right breast in women with developed breasts; the other was a slanted incision for men and children. These surgical procedures took place between July 1996 and November 1997. Intraoperatively, a right atriotomy was used to repair 11 atrial septal defects and 11 ventricular septal defects, 2 combined atrial and ventricular septal defects, 1 case of a single atrium, and 1 partial atrioventricular canal. A right ventricular outflow tract incision was used to repair 7 ventricular septal defects and 7 ruptured aortic sinus aneurysm. A combination of a right atriotomy and right ventricular outflow tract incision was used for 2 repairs of combined atrial and ventricular septal defects, 3 radical corrections of tetralogy of Fallot, and 2 radical corrections of trilogy of Fallot. A combined right and interatrial septal incision was used for 6 mitral valve replacements and 1 mitral valvuloplasty. Smooth bypass cannulation and satisfactory intracardiac exposure were achieved with the right anterolateral thoracotomy. There was no complication or mortality directly related to the incision. We believe that the right anterolateral thoracotomy is safer and more effective than the median sternotomy for many common congenital and acquired heart diseases. The thoracotomy causes less trauma and results in a cosmetic appearance that is more acceptable to the patient. (+info)
Complete atrioventricular septal defect associated with tetralogy of fallot: surgical indications and results.
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Complete atrioventricular septal defect (AVSD) associated with tetralogy of Fallot is a rare condition that still has problems in the postoperative period. The authors report their surgical experiences over the past 10 years. Nine children underwent total correction. The defect was repaired by the 2-patch technique and the ATrioventricular valve was reconstructed by suturing the cleft and annuloplasty. A transannular right ventricular outflow patch was used in 5 patients. All patients had Down syndrome and a free-floating superior bridging lEAflet. One patient died from cardiac failure. Although there was no reoperation or death in the late postoperative periods, mild mitral regurgitation occurred in 4 patients and there was moderate or severe pulmonary regurgitation in 2 patients. All survivors currently have no critical symptoms in their daily lives. With the standard of patient selection used, the optimal body weight was around 8 kg and PA index was 200 or more. Right ventriculotomy provided a better view for complete closure of the ventricular septal defect (VSD). In order to avoid re-regurgitation of the atrioventricular valve, the 2-patch technique is the most suitable procedure for total repair. (+info)
Pulmonary atresia with intact ventricular septum percutaneous radiofrequency-assisted valvotomy and balloon dilation versus surgical valvotomy and Blalock Taussig shunt.
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OBJECTIVE: We compared the result of radiofrequency (RF)-assisted valvotomy and balloon dilation with closed surgical valvotomy and Blalock Taussig (BT) shunt as primary treatment in selected patients with pulmonary atresia and intact ventricular septum (PA-IVS). BACKGROUND: Patients with PA-IVS who have mild to moderate hypoplasia of the right ventricle (RV) and patent infundibulum have the greatest potential for complete biventricular circulation. The use of RF or laser wires to perforate the atretic valve followed by balloon dilation provides an alternative to surgery. METHODS: Between May 1990 and March 1998, 33 selected patients underwent either percutaneous RF valvotomy and balloon dilation (group 1, n = 21; two crossed over to group 2) or surgical valvotomy with concomitant BT shunt (group 2, n = 14). Second RV decompression by balloon dilation or right ventricular outflow tract (RVOT) reconstruction were performed if necessary. Patients who remained cyanosed were subjected to transcatheter trial closure of the interatrial communication. Partial biventricular repair was offered to those with inadequate growth of the RV. RESULTS: The primary procedure was successful in 19 patients in group 1. There was one in-hospital death and two late deaths. Of the remaining 16 survivors, 12 achieved complete biventricular circulation, 7 of whom required no further interventions. Two patients required repeat balloon dilation, 1 RVOT reconstruction and 2 transcatheter closure of interatrial communication. Two patients underwent partial biventricular repair. In group 2, there were 3 in-hospital deaths after the primary procedure and 1 patient died four months later. All survivors (n = 10) required a second RV decompression, 8 by balloon dilation and 2 by RVOT reconstruction, after which, two patients died. Of the final 8 survivors, 7 achieved complete biventricular circulation, 5 after coil occlusion of the BT shunt and 2 after closure of interatrial communication. CONCLUSIONS: Radiofrequency valvotomy and balloon dilation is more efficacious and safe compared with closed pulmonary valvotomy and BT shunt in selected patients with PA-IVS. (+info)