Search

A search of peer-reviewed literature was conducted to identify reports that provide data on complications associated with pregnancy in women with structural congenital heart disease (CHD). This review describes the outcome of 2,491 pregnancies, including 377 miscarriages (15%) and 114 elective abortions (5%). Important cardiac complications were seen in 11% of the pregnancies. Obstetric complications do not appear to be more prevalent. In complex CHD, premature delivery rates are high, and more children are small for gestational age. The offspring mortality was high throughout the spectrum and was related to the relatively high rate of premature delivery and recurrence of CHD.

Objectives: This study sought to compare the safety and efficacy of the HELEX septal occluder (HSO) with surgical repair of atrial septal defect (ASD)., Background: The HSO is a low-profile, double-disk occluder device for percutaneous closure of secundum ASD., Methods: Patients were enrolled (HSO arm prospectively, surgery arm prospectively/retrospectively) from 14 U.S. sites and followed up for 12 months postprocedure. Investigator-reported outcomes were evaluated, including closure success (no or clinically insignificant residual shunt) and the incidence of adverse events. The first 3 HSO patients at each site were considered training cases and were excluded from analysis., Results: Between March 2001 and April 2003, 119 nontraining cases received an HSO and 128 had surgical repair. The groups were similar with statistical but clinically unimportant differences in median age, weight, and preprocedural echocardiographic defect size. Anesthesia time and hospital stay were significantly shorter in the HSO group. Closure success, defined as complete closure or a clinically insignificant residual shunt, was similar in both groups. Major and minor adverse events rates were not statistically different. The most common major adverse events for the HSO group was device embolization requiring catheter retreival (1.7%), and in the surgery group was postpericardiotomy syndrome (6.3%), including one death because of tamponade. The primary end point, clinical success, a composite of closure success and no major adverse events at 12 months, satisfied the noninferiority hypothesis comparing device closure with surgery., Conclusions: Closure of ASD with the HELEX septal occluder is safe and effective when compared with surgical repair, with reduced anesthesia time and hospital stay. (U.S. Multicenter Pivotal Study of the HELEX Septal Occluder for Percutaneous Closure of Secundum Atrial Septal Defects; this study was approved by the Food and Drug Administration before the National Institutes of Health website was active, so there is not a URL or registration number.).

Objectives: We sought to determine the association between patent foramen ovale (PFO), atrial septal aneurysm (ASA), and stroke prospectively in a unselected population sample., Background: The disputed relationship between PFO and stroke reflects methodologic weaknesses in studies using invalid controls, unblinded transesophageal echocardiography examinations, and data that are unadjusted for age or comorbidity., Methods: The use of transesophageal echocardiography to identify PFO was performed by a single echocardiographer using standardized definitions in 585 randomly sampled, Olmsted County (Minnesota) subjects age 45 years or older participating in the Stroke Prevention: Assessment of Risk in a Community (SPARC) study., Results: A PFO was identified in 140 (24.3%) subjects and ASA in 11 (1.9%) subjects. Of the 140 subjects with PFO, 6 (4.3%) had an ASA; of the 437 subjects without PFO, 5 had an ASA (1.1%, two-sided Fisher exact test, p = 0.028). During a median follow-up of 5.1 years, cerebrovascular events (cerebrovascular disease-related death, ischemic stroke, transient ischemic attack) occurred in 41 subjects. After adjustment for age and comorbidity, PFO was not a significant independent predictor of stroke (hazard ratio 1.46, 95% confidence interval 0.74 to 2.88, p = 0.28). The risk of a cerebrovascular event among subjects with ASA was nearly four times higher than that in those without ASA (hazard ratio 3.72, 95% confidence interval 0.88 to 15.71, p = 0.074)., Conclusions: These prospective population-based data suggest that, after correction for age and comorbidity, PFO is not an independent risk factor for future cerebrovascular events in the general population. A larger study is required to test the putative stroke risk associated with ASA.

Objectives: We sought to analyze the morphologic and functional characteristics of the patent foramen ovale in patients with different clinical likelihoods for paradoxic embolism., Background: The incidence of patent foramen ovale is increased in patients with otherwise unexplained arterial ischemic events. Because signs of venous thrombosis are absent in most patients, the diagnosis of paradoxic embolism is often questioned, even when patent foramen ovale is the only potential explanation for the ischemic event., Methods: Seventy-eight patients with a patent foramen ovale detected by contrast transesophageal echocardiography were studied: 21 patients with an otherwise unexplained arterial ischemic event and clinical evidence implying paradoxic embolism (group I), 30 patients with an unexplained ischemic event but no clinical evidence for paradoxic embolism (group II) and 27 patients without an ischemic event (group III)., Results: During transesophageal contrast echocardiography, patients in group I had more severe right to left shunting (mean +/- SD 52 +/- 16% of the left atrial area filled with contrast medium) and a wider opening of the patent foramen ovale (7.1 +/- 3.6-mm separation between the septum primum and the septum secundum) than did patients in group II (35 +/- 15% and 4.4 +/- 3.2 mm, respectively, p < 0.001) or group III (23 +/- 12% and 3.0 +/- 2.0 mm, respectively, p < 0.001). The incidence of atrial septal aneurysm was similar in the three groups. Severe contrast shunting (> or = 50% of the left atrial area filled with contrast medium) and wide opening of the patent foramen ovale (> or = 5-mm separation) revealed a high sensitivity (71% and 86%, respectively) and high specificity (86% and 96%, respectively) for identification of group I patients., Conclusions: Right to left contrast shunting is more severe and opening of the patent foramen ovale is larger in patients with ischemic arterial events considered to be due to paradoxic embolism. In patients with a patent foramen ovale as the only potential cause for ischemic events and no signs of venous thrombosis, morphologic and functional variables assessed by transesophageal echocardiography may be helpful in estimating the likelihood of paradoxic embolism.

Objectives: We postulated that femoral vein delivery of contrast medium because of streaming, might enhance precordial echocardiographic detection of patent foramen ovale., Background: Although precordial contrast echocardiography is widely used to diagnose patent foramen ovale, this method is limited by poor sensitivity. Previous investigators have demonstrated enhanced detection of atrial defects by the dye-dilution technique after delivery of contrast medium into the inferior rather than the superior vena cava., Methods: Transthoracic contrast examinations were performed in a randomly selected group of 70 patients (without previous history of cerebral or systemic embolus) undergoing cardiac catheterization. Paired contrast agent injections (10 ml dextrose in water/0.25 ml air) were administered from an upper extremity vein and femoral vein in each patient during spontaneous respiration, cough and Valsalva maneuvers. Studies were interpreted by an experienced echocardiographer unaware of the sequence and site of injections. Positive studies were semiquantitatively graded from +1 (minimal left ventricular opacification) to +4 (intense left ventricular opacification). Catheterization and echocardiographic assessment of patent foramen ovale were compared in 21 subjects., Results: Patent foramen ovale was detected significantly more often during femoral vein versus upper extremity contrast delivery (23 of 70 patients [prevalence 33%] vs. 9 of 70 patients [prevalence 13%], p < 0.001). The intensity of left ventricular opacification was also greater during femoral vein contrast injection. Precordial echocardiography combined with femoral contrast delivery was significantly more sensitive than cardiac catheterization for assessment of patent foramen ovale (8 of 21 patients vs. 2 of 21 patients, p < 0.05)., Conclusions: Femoral vein contrast delivery significantly enhances the ability of precordial contrast echocardiography to diagnose patent foramen ovale. Physiologic patency of the foramen ovale is more common (prevalence 33%) than previously documented.

Objectives: The purpose of this study was to determine the incidence of cardiologic abnormalities in Noonan syndrome., Background: The incidence of cardiac abnormalities in Noonan syndrome remains unknown, largely because of such difficulties as assembling a substantial cohort, ensuring a correct phenotypic diagnosis and providing accurate definitions of the most frequent abnormalities--pulmonary stenosis and left ventricular hypertrophy., Methods: A cohort of 145 patients was assembled, and before cardiologic assessment two independent geneticists scrutinized the phenotype. The diagnosis was confirmed in 118 patients, and they were studied by two-dimensional and Doppler echocardiography., Results: A dysplastic pulmonary valve was present in eight patients (7%) and was associated with significant stenosis in six (75%) of the eight. Significant stenosis was present in 22 (20%) of 110 patients without dysplasia. Left ventricular hypertrophy was present in 29 patients (25%) without significant pulmonary stenosis. Localized anterior septal hypertrophy was the most common pattern in 12 (41%) of 29 patients. Diffuse hypertrophy involving the entire septum and the free wall was present in nine patients (31%) and was severe (> 1.7 cm) in five. Other abnormalities included secundum atrial septal defects (10%)., Conclusions: The high incidence of cardiac abnormalities suggests that echocardiographic and Doppler evaluation of patients with the Noonan phenotype is important because it will aid in genetic counseling and in the assessment of the natural history of--and, ultimately, identification of the gene(s) responsible for--Noonan syndrome.

Objectives: To establish the rate of spontaneous closure of atrial septal defects diagnosed before age 3 months, 101 infants (mean age 26 days) with an interatrial shunt confirmed by Doppler echocardiography were followed up for an average of 265 +/- 190 days., Background: Even if interatrial shunts in the newborn are frequently encountered, little is known about their natural history., Methods: Defect diameter on two-dimensional echocardiography and width of color flow jet were measured in the subcostal view. Right and left ventricular diameters and atrial septal curvature were also studied. Kaplan-Meier curves were obtained to predict age of spontaneous closure in relation to initial defect diameter., Results: There was no significant correlation between the diameter of the atrial septal defect and right ventricular/left ventricular ratio or type of septal curvature (vertical or concave toward the left atrium). The classic predominance of girls over boys was observed only for defects > 5 mm. An overall rate of spontaneous closure of 87% was observed. Frequency and timing of closure were inversely correlated to atrial septal defect diameter: closure occurred in 100% (32 of 32) of defects in group 1 (diameter < 3 mm), 87% of defects (39 of 45) in group 2 (diameter 3 to 5 mm), 80% of defects (16 of 20) in group 3 (diameter 5 to 8 mm). Spontaneous closure did not occur in four patients of group 4 (defect > or = 8 mm) during an average follow-up interval of 417 days (range 294 to 597 days)., Conclusions: These results suggest that infants with an atrial septal defect < 3 mm need not be followed up as 100% of these defects will be closed by age 18 months; those with a defect 3 to 5 or 5 to 8 mm should be evaluated by the end of the 12th and the 15th month, respectively, when > 80% of these defects will be closed. An atrial septal defect with a diameter > or = 8 mm may have little chance of closing spontaneously and the possibility of surgical correction should be considered. Defects < 3 mm probably do not constitute a cardiac malformation in light of their natural evolution and gender distribution.

Objectives: The purpose of this study was to evaluate the ability of velocity-encoded nuclear magnetic resonance (NMR) imaging to quantify left to right intracardiac shunts in patients with an atrial septal defect., Background: Quantification of intracardiac shunts is clinically important in planning therapy., Methods: Velocity-encoded NMR imaging was used to quantify stroke flow in the aorta and in the main pulmonary artery in a group of patients who were known to have an increased pulmonary to systemic flow ratio (Qp/Qs). The velocity-encoded NMR flow data were used to calculate Qp/Qs, and these values were compared with measurements of Qp/Qs obtained with oximetric data derived from cardiac catheterization and from stroke volume measurements of the two ventricles by using volumetric data from biphasic spin echo and cine NMR images obtained at end-diastole and end-systole., Results: Two independent observers measured Qp/Qs by using velocity-encoded NMR imaging in 11 patients and found Qp/Qs ranging from 1.4:1 to 3.9:1. These measurements correlated well with both oximetric data (r = 0.91, SEE = 0.35) and ventricular volumetric data (r = 0.94, SEE = 0.30). Interobserver reproducibility for Qp/Qs by velocity-encoded NMR imaging was good (r = 0.97, SEE = 0.20)., Conclusions: Velocity-encoded NMR imaging is an accurate and reproducible method for measuring Qp/Qs in left to right shunts. Because it is completely noninvasive, it can be used to monitor shunt volume over time.

Objectives: The purpose of this study is to determine the early and late results of the surgical repair of atrial septal defect in adults., Background: Progressively limiting, untreated atrial septal defect can lead to the early death of middle-aged adults. Recently it has been suggested that the closure of atrial septal defects might be accomplished with interventional cardiac techniques. Although the long-term results of the transcatheter closure are as yet unknown, the outcome of surgical therapy has been shown to be beneficial for almost 40 years., Methods: Between 1971 and 1991, 166 consecutive patients underwent surgical repair of a secundum or sinus venosus atrial septal defect, or both, at the Brigham and Women's Hospital, Boston. There were 120 women and 46 men in this group; the mean age was 44 years and 58 (35%) of the patients were > or = 50 years old. The average pulmonary to systemic flow ratio was 3.0, and 57 patients had a peak systolic pulmonary artery pressure > 30 mm Hg., Results: There were two operative deaths (early mortality rate 1.2%), and 13% of the patients had a perioperative complication. One hundred fifty-three of the 164 survivors were followed up for a mean of 90 months (range 2 to 247). There were eight late deaths (late mortality rate 4.9%) and a late morbidity rate of 12.4% (in most cases due to arrhythmias). The 5- and 10-year survival rates are 98% and 94%, respectively, and the probability of event-free survival (with no morbidity or mortality) at 5 years is 97% and at 10 years is 92%., Conclusions: The results indicate that the surgical correction of atrial septal defect in adults is safe and efficacious as confirmed by 20 years of follow-up.

Detection of patent foramen ovale by contrast echocardiography is based on transient inversion (right atrial pressure higher than left atrial pressure) of the interatrial pressure gradient. Therefore, the presence of left-sided heart disease with potential elevation of left atrial pressure might obscure the diagnosis of patent foramen ovale. Accordingly, 150 patients (88 men, 62 women; mean age 51.7 +/- 15.2 years) were evaluated for a patent foramen ovale by transesophageal contrast echocardiography. Additionally, atrial septal motion during normal respiration and during the Valsalva maneuver was analyzed. Patency of the foramen ovale was observed in 20 (27%) of 74 patients without left-sided heart disease and with previous arterial embolism, in none (0%) of 25 patients with left-sided heart disease and embolism, in 7 (39%) of 18 patients without left-sided heart disease and without embolism and in 3 (9%) of 33 patients with left-sided heart disease and without embolism. The detection rate of patent foramen ovale was lower in patients with than without left-sided heart disease (5% vs. 29%, p = 0.0007) but was similar in patients with and without embolism (20% vs. 19.5%, p = NS). Abnormal atrial septal motion was more frequently observed in patients with left-sided heart disease (p = 0.0003) and was inversely correlated to detection of patent foramen ovale (p = 0.0003). Multivariate analysis revealed an independent association between the absence of left-sided heart disease and the detection of patent foramen ovale (p = 0.0003). These data suggest that in patients with left-sided heart disease, patency of the foramen ovale may be missed even by transesophageal contrast echocardiography.

In atrioventricular (AV) septal defect, the common AV valve can have a common orifice or can be divided by bridging leaflet tissue into two separate orifices. To determine the accuracy of a two-dimensional echocardiographic technique devised specifically for evaluation of the number of AV valve orifices, all 69 children undergoing surgical repair of AV septal defect from April 1987 to August 1990 were examined prospectively. The presence of bridging leaflet tissue and the number of AV valve orifices were determined with use of a subcostal imaging plane. From a standard subcostal four-chamber view, the plane of sound was rotated 30 degrees to 45 degrees clockwise until the AV valve was seen en face. The plane of sound was then tilted from a superior to an inferior direction so that cross-sectional views of the AV valve were examined from the inferior margin of the atrial septum to the superior margin of the ventricular septum. Of the 69 patients, 6 (9%) were excluded because the appropriate subcostal images were not obtained (in 3 because of obesity and in 3 as a result of operator failure). The remaining 63 children, ranging in age from 1 day to 13.5 years and in weight from 1 to 55 kg, constituted the study group. Echocardiographic results were compared with surgical observations in 62 patients and with autopsy findings in 1 patient. With the two-dimensional echocardiographic technique, 32 of 33 patients with a common orifice and 28 of 30 patients with two separate AV valve orifices were correctly identified.(ABSTRACT TRUNCATED AT 250 WORDS)

Five variations of atrial septal morphology occur in hypoplastic left heart syndrome. One variety, termed anomalous attachment of septum primum, has been described only in necropsy series. Two-dimensional echocardiography was utilized to determine the incidence of this anomaly in patients with left atrioventricular (AV) valve underdevelopment, including those with other ventriculoarterial alignments, such as transposition of the great arteries. Forty-eight (37%) of 129 patients with normally aligned great arteries (and two ventricles) had anomalous attachment of septum primum. Ten (34%) of 29 patients with double outlet right ventricle and left AV valve underdevelopment had this anomaly. Four (50%) of eight patients with single ventricle exhibited this atrial septal variant. The most reliable view to identify anomalous attachment was the subcostal left oblique-equivalent cut. Recognition of atrial septal morphology has implications for preoperative and intraoperative management of patients with left AV valve underdevelopment. The similar prevalence of this atrial septal variant in patients with normally aligned great arteries, double outlet right ventricle and transposed great arteries suggests that there may be a common mechanism for left AV valve underdevelopment that is independent of the development of the arterial portion of the heart.

The effect of high altitude on the prevalence of congenital heart disease was tested by examining 1,116 school children at four study sites in the People's Republic of China. Sites ranged in altitude from sea level to 4,500 m above sea level. Children were screened by physical examination, and an echocardiogram and electrocardiogram were performed on each child suspected of having a cardiac anomaly. A high prevalence of patent ductus arteriosus and atrial septal defect was found at the three high altitude sites and the effect of altitude was progressive. Both anomalies were postulated to be the result of the lower atmospheric oxygen tension present at high altitude. Failure of lower oxygen tension to constrict the ductus is thought to be the mechanism in patent ductus arteriosus. It is theorized that the persistence of high pulmonary vascular resistance and high right heart pressures at high altitude inhibits early closure of the foramen ovale. Subsequent growth may result in stretching of the fossa ovalis and incompetence of the flap and may produce an atrial septal defect. The high prevalence of atrial septal defect in tetralogy of Fallot is cited as a possible analogy because right ventricular pressure is high and right ventricular compliance is low from birth.