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The spectrum of adult congenital heart defects seen in echocardiography practice varies according to institutional practice and expertise. Half a century ago, survival with severe congenital heart disease was less common. Today, nearly 80% of such patients in industrialized societies now survive into adulthood. Most are followed up in centers that specialize in adult congenital heart diseases (CHDs), but it is not uncommon for such adults to be seen in general echocardiography practice.

Objectives: The CardioSEAL VSD registry was created to track safety of the device to close high-risk Ventricular Septal Defect (VSD). Background: This is the first report from the multi-centered CardioSEAL VSD registry reviewing demographics and initial results. Methods: Centers recruited patients with VSD who were high-risk for surgery due to medical condition or anatomic features. Results: 18 centers contributed data on 55 high-risk patients who had 61 VSD-occlusion procedures, with age of range of 5 days to 65 years and using one to six devices. Implantation approach was transvenous in 48, perventricular in five, and by combined approach in two patients. Ninety-two percent of intended VSD device implants were judged successful. Twenty-two patients had single VSD closed by single device in 18 and by two devices in four patients. All patients

Improved medical and surgical therapies for children with congenital heart disease have resulted in a growing population of patients reaching adulthood.1,2 Over this same time, the field of interventional cardiology has experienced significant growth, driven by technological improvements and better understanding of the mechanisms and intermediate-term results of individual procedures. Consequently, for adults with uncorrected or previously palliated congenital heart disease, percutaneous therapies have increasing acceptance as reasonable additions, alternatives, and treatments of choice when further surgical or medical intervention is contemplated (Table 1). Currently, interventional cardiology of adult congenital heart disease (ACHD) is a well-established field on its own and, programmatically, is a fundamental component of any center providing care for these patients.3 Although limited safety of interventional procedures for the ACHD population has been suggested in centers without ACHD global care programs,4 the wide variation in clinical presentation, novelty of cardiovascular pathologies (with similarity to as well as marked differences from both congenital and acquired conditions), and potential for concomitant multiple organ system pathology all contribute to situations atypical for standard adult or pediatric laboratories. These concerns, combined with a desire to centralize data collection to establish outcomes assessments for ACHD patients considered for interventional catheterization, lead to the recommendation that such procedures be performed in centers with ACHD expertise and established care programs.3 View this table: TABLE 1. Commonly Practiced Catheter-Based Interventions in ACHD: Indications and Level of Evidence In this article, we review the most commonly performed percutaneous procedures in ACHD, including valvuloplasty, angioplasty, and device closures. We also include discussions concerning complex ACHD patients (Table 2) as well as future directions. View this table: TABLE 2. Potential Interventions in Complex ACHD Patients ### Pulmonary Valve Stenosis Pulmonary valve stenosis (PS) is almost always congenital in origin and usually results from commissural fusion of thin and pliable leaflets. Less frequently, the pulmonary …

Complications with extraction of abundant endovascular systems increase with time since implantation. As the number of implanted devices increases, successful management of complications needs to be disseminated. We present a 46-year-old woman with endovascular leads placed 15 years previously requiring extraction. Using laser-assistance the leads were removed, although the passive lead tips were unable to be extracted, and were retained in the superior vena cava. One lead tip embolized to the distal pulmonary bed within 24 hours of her operative procedure. Computed tomography and pulmonary arteriography suggested a near immediate thrombogenic process. A multidisciplinary approach was utilized to identify management strategies that allowed for a satisfactory patient outcome.

Background: Stenting for aortic coarctation has been shown to be effective in the short term. The safety and longer term efficacy of transcatheter therapy, however, must be well established if the technique is to be widely accepted as an alternative to surgery. In order to determine the frequency, spectrum, and outcome of injury to the aortic wall caused by angioplasty or stenting of aortic coarctation, the nomenclature of mural injury in these patients must be adapted to the conditions of transcatheter therapy. Methods and Results: Between 1989 and July 2005, we inserted stents in 153 patients with aortic coarctation, their median age being 15.8 years. Prior aortic interventions had been performed in 98 patients, and preexisting aneurysms were observed in 19. Stenting resulted in a significant reduction of the gradient across the site of coarctation, from a median of 30 millimetres of mercury to zero (p less than 0.001), with a residual gradient within the aortic arch of 20 millimetres of mercury or more in 5% of patients. Acute injuries to the aortic wall, other than therapeutic tears, were observed in 3 patients (2%), none of whom required surgery. At median follow-up of 2.5 years, this being more than 5 years in 30 patients, 4 patients had died, albeit none from complications relating to stenting or catheterization. Acute injuries to the aortic wall did not progress, and new aneurysms were observed in 6% of patients subsequent to follow-up imaging. Stent fractures, and jailed or partially covered brachiocephalic vessels, were observed in 12, and 49, patients, respectively, but did not result in haemodynamic or embolic complications. Conclusions: Stenting for aortic coarctation results in consistent relief of the gradient, and few serious complications in the short and intermediate term. Serious injuries to the aortic wall are uncommon in our experience, and can be minimized with a focus on technical measures, such as pre-dilation before stenting.

In the new Congenital Heart Disease and Pediatric Cardiology subsection, 186 abstracts were submitted to the Program Committee of the 55th Annual Scientific Session, with 60 accepted and presented during 8 abstract sessions. In addition, there were 17 structured sessions at ACC.06. Altogether, 50

Increasingly, women born with complete transposition of the great arteries who have undergone atrial repair by either the Senning or the Mustard procedure are reaching childbearing age. This study reports on pregnancy outcomes after the atrial repair of transposition of the great arteries. Record review and standardized questionnaires were used to ascertain the outcomes of 70 pregnancies reported in 40 women (36 Mustard procedures, 4 Senning procedures). Of the 70 pregnancies, 54 resulted in 56 live births, 10 in miscarriages, and 6 in therapeutic abortions. At pregnancy, 31 women were in New York Heart Association class I, 8 were in class II, and 1 was in class III. Thirty-nine percent of the infants were delivered prematurely and weighed 2,714 +/- 709 g; 28% were delivered by cesarean section, 8 for cardiac indications. Maternal complications included arrhythmias in 5 women and hemoptysis in 2 women. Heart failure occurred in 6 women, developing during the second and third trimesters. Postpartum cardiac events developed 2 to 9 days postpartum: heart failure in 5 women, atrial fibrillation in 1 woman, and decreased oxygen saturation due to a new atrial baffle leak in 1 woman. Severe right ventricular (RV) failure led to cardiac transplantation after delivery in 1 woman; another developed heart failure and then died suddenly 1 month after delivery. There was 1 late death, 4 years after the patient's last pregnancy. In conclusion, pregnancy after atrial repair carries a moderate degree of risk and should be undertaken with caution.

Post utero, persistence of the patent foramen ovale (PFO) carries mischievous potential, as it appears to have no physiologic or survival benefit for normal individuals. In states of abnormal right-sided cardiac capacitance, PFO has been implicated in worsening hypoxemia resulting from right to left intracardiac passage of deoxygenated blood. Association and causative roles of PFO in much more commonly occurring syndromes, including cerebral and cutaneous decompression disease, migraine with aura, and embolic ischemic stroke, have been suggested.

To the Editor: Of the approximately 1.3 million adult survivors with congenital heart disease (CHD) living in the United States, only a small percentage appears to be receiving specialized care in accordance with the American College of Cardiology (ACC) 2008 Guidelines for the Management of Adult

Guidelines for the successful orchestration of transitioning of the adolescent and young and older adult patient with congenital heart disease to a health care system appropriate for their long-term congenital heart disease care and counseling appear necessary to improve patient and family confidence, education, therapy, life quality, and survival outcomes. Schema for care organization and delivery for adult patients with congenital heart disease remain primitive and largely unimplemented. The presence of a strong central care oversight organization and the establishment of a multi-institutional database to assist in assessment of care outcomes and guidelines appears requisite to these needs and for the establishment of transitioning guidelines for these patients as they assume a greater and deeper shared control of their futures with their caregivers.

Background— Our purpose was to describe a 13-year experience with patients undergoing transcatheter device closure of unrepaired congenital or postoperative residual ventricular septal defects (VSDs). Methods and Results— Since 1989, 170 patients (median age, 3.9 years) have undergone catheterization for closure of 1 or more congenital (n=92) or postoperative (n=78) residual VSDs using successive generations of STARFlex-type devices. Outcomes included echocardiographic assessment of residual flow and device position, assessment of VSD shunt/severity, and adverse events. Among 168 patients in whom device implantation was performed, between 1 and 7 devices were placed per patient (median, 1), with multiple devices placed in 40%. There was a significant decrease in left-to-right shunting after device implantation ( P Conclusions— Congenital and postoperative VSD closure using STARFlex-type devices resulted in stable improvement in clinical status and decreased interventricular shunting. Although periprocedural events occurred frequently, late events caused by the device were rare. Transcatheter device closure is an effective management option for patients with complex muscular VSDs that are difficult to approach surgically and for postoperative residual VSDs.

Background The optimal strategy to prevent recurrent presumed paradoxical emboli in patients with patent foramen ovale is unknown. Purpose To synthesize the current knowledge about and qualitatively assess the relative benefits of transcatheter closure versus medical therapy for patent foramen ovale. Data sources English-language and foreign-language journals listed in the MEDLINE database from January 1985 to July 2003 were systematically searched. Secondary sources were also used. Study selection Secondary prevention studies of transcatheter closure or medical therapy for patent foramen ovale were required to include at least 10 patients followed for more than 1 year and to report recurrent neurologic events. Data extraction Data from published studies were manually extracted and summarized. Data synthesis Ten studies of transcatheter closure (1355 patients) and 6 studies of medical therapy (895 patients) for patent foramen ovale were included. Overall, the 1-year rate of recurrent neurologic thromboembolism with transcatheter intervention was 0% to 4.9%, and the incidence of major and minor complications was 1.5% and 7.9%, respectively. Medical management was associated with a 1-year recurrence rate of 3.8% to 12.0%. However, limitations resulting from uncontrolled data, nonstandardized definitions, vigilance of follow-up, and baseline imbalances preclude definitive conclusions about the superiority of a particular approach. General differences in study samples included older age, greater proportion of men, and higher prevalence of diabetes and smoking among medically treated patients. Patients undergoing treatment with a transcatheter device were more likely to have had multiple thromboembolic events at baseline. Conclusion Transcatheter closure of patent foramen ovale may prevent a substantial proportion of cryptogenic strokes. Randomized clinical trials are needed.

Ventricular septal defect with intramyocardial dissection of the ventricular free wall is a rare complication of myocardial infarction associated with poor prognosis. We describe a patient who developed a ventricular septal defect with intramyocardial dissection of the right ventricular free wall. Initially the patient was successfully stabilized by the placement of a percutaneous closure device. The placement of the device allowed initial hemodynamic recovery of the patient and subsequent definitive surgical repair. This case illustrates the importance of collaboration between interventional cardiologists and cardiac surgeons in the treatment of complex postinfarction ventricular septal defects.

Major orthopaedic procedures that involve instrumentation of the medullary canal are well known to produce showers of embolic debris including fat, bone fragments, marrow constituents, and cement. A growing literature has identified paradoxic cerebral embolism as a rare but serious consequence of the passage of these emboli through an intracardiac right-to-left shunt to the cerebral arterial circulation. We report 2 cases of paradoxic cerebral embolism after cemented total knee arthroplasty and propose a novel treatment plan to prevent recurrent stroke during subsequent surgery.

Background — In patients with complex pulmonary stenosis or atresia, a detailed delineation of all sources of pulmonary blood supply is necessary for planning surgical and transcatheter procedures and usually requires diagnostic cardiac catheterization. The goals of this study were to determine whether gadolinium-enhanced 3D magnetic resonance angiography (MRA) can provide a noninvasive alternative to diagnostic catheterization and to compare MRA and x-ray angiography measurements of pulmonary arteries and aortopulmonary collaterals (APCs). Methods and Results — Thirty-two patients with pulmonary stenosis or atresia (median age: 4.7 years, range: 1 day to 46.9 years) underwent both MRA and cardiac catheterization (median time: 1 month). Diagnoses included tetralogy of Fallot (TOF) with pulmonary atresia (n=13), TOF with pulmonary stenosis (n=4), post-Fontan palliation (n=5), and other complex congenital heart disease (n=10). Compared with catheterization and surgical observations, MRA had a 100% sensitivity and specificity for the diagnosis of main (n=10) and branch pulmonary artery (PA) stenosis or hypoplasia (n=38), as well as absent (n=5) or discontinuous (n=4) branch PAs. All 48 major APCs diagnosed by catheterization were correctly diagnosed by MRA. Three additional APCs were diagnosed by MRA but not by catheterization. The mean difference between MRA and catheterization measurements of 33 pulmonary vessel diameters was 0.5±1.5 mm, with a mean interobserver difference of 0.4±1.5 mm. Conclusions — Gadolinium-enhanced 3D MRA is a fast and accurate technique for delineation of all sources of pulmonary blood supply in patients with complex pulmonary stenosis and atresia and can be considered a noninvasive alternative to diagnostic catheterization with x-ray angiography.

Endothelin-1 is a potent vasoconstrictor and smooth-muscle mitogen. In a preliminary study, the orally administered dual endothelin-receptor antagonist bosentan improved exercise capacity and cardiopulmonary hemodynamics in patients with pulmonary arterial hypertension. The present trial investigated the effect of bosentan on exercise capacity in a larger number of patients and compared two doses.In this double-blind, placebo-controlled study, we randomly assigned 213 patients with pulmonary arterial hypertension (primary or associated with connective-tissue disease) to receive placebo or to receive 62.5 mg of bosentan twice daily for 4 weeks followed by either of two doses of bosentan (125 or 250 mg twice daily) for a minimum of 12 weeks. The primary end point was the degree of change in exercise capacity. Secondary end points included the change in the Borg dyspnea index, the change in the World Health Organization (WHO) functional class, and the time to clinical worsening.At week 16, patients treated with bosentan had an improved six-minute walking distance; the mean difference between the placebo group and the combined bosentan groups was 44 m (95 percent confidence interval, 21 to 67; P0.001). Bosentan also improved the Borg dyspnea index and WHO functional class and increased the time to clinical worsening.The endothelin-receptor antagonist bosentan is beneficial in patients with pulmonary arterial hypertension and is well tolerated at a dose of 125 mg twice daily. Endothelin-receptor antagonism with oral bosentan is an effective approach to therapy for pulmonary arterial hypertension.

Objective: To assess short-term and long-term responses to treatment with pulmonary vasodilators in patients with sarcoidosis-related pulmonary hypertension. Methods: A prospective, observational study was performed on eight patients with moderate-tosevere sarcoidosis-related pulmonary hypertension. Patients underwent a short-term vasodilator trial, using inhaled nitric oxide (iNO), IV epoprostenol, and/or oral calcium-channel blockers. A favorable short-term response was considered a > 20% decrease in pulmonary vascular resistance (PVR). Five patients received long-term treatment with iNO (with one patient receiving epoprostenol in addition) and underwent follow-up hemodynamic and/or 6-min walk testing. Two patients received long-term treatment with calcium-channel blockers. Results: Baseline ( SE) mean pulmonary artery pressure (mPAP) was 55 4 mm Hg and PVR was 896 200 dynescm 5 . A favorable short-term response was seen in seven of eight patients receiving iNO, four of six patients receiving epoprostenol, and two of five patients receiving calcium-channel blockers. With iNO, PVR decreased 31 5% (p 0.006) and mPAP decreased 18 4% (p 0.003); with epoprostenol, PVR decreased 25 6% (p 0.016) and mPAP decreased 6 2% (p not significant). Decreased systemic vascular resistance was the only significant response to treatment with calcium-channel blockers. Follow-up 6-min walk test results improved in all five patients receiving long-term treatment with iNO. Follow-up hemodynamic responses in three patients showed preserved vasoresponsiveness. These three patients subsequently died, as did the two patients receiving calcium-channel blockers. The two remaining patients continue to receive iNO. Conclusion: In the short term, pulmonary hypertension in patients with sarcoidosis is responsive to treatment with pulmonary vasodilators; these patients may benefit from long-term iNO therapy. (CHEST 2001; 120:866 – 872)

OBJECTIVES We sought to characterize re-entry circuits causing intra-atrial re-entrant tachycardias (IARTs) late after the repair of congenital heart disease (CHD) and to define an approach for mapping and ablation, combining anatomy, activation sequence data and entrainment mapping. BACKGROUND The development of IARTs after repair of CHD is difficult to manage and ablate due to complex anatomy, variable re-entry circuit locations and the frequent co-existence of multiple circuits. METHODS Forty-seven re-entry circuits were mapped in 20 patients with recurrent IARTs refractory to medical therapy. In the first group (n = 7), ablation was guided by entrainment mapping. In the second group (n = 13), entrainment mapping was combined with a three-dimensional electroanatomic mapping system to precisely localize the scar-related boundaries of re-entry circuits and to reconstruct the activation pattern. RESULTS Three types of right atrial macro–re-entrant circuits were identified: those related to a lateral right atriotomy scar (19 IARTs), the Eustachian isthmus (18 IARTs) or an atrial septal patch (8 IARTs). Two IARTs originated in the left atrium. Radiofrequency (RF) lesions were applied to transect critical isthmuses in the right atrium. In three patients, the combined mapping approach identified a narrow isthmuses in the lateral atrium, where the first RF lesion interrupted the circuit; the remaining circuits were interrupted by a series of RF lesions across a broader path. Overall, 38 (81%) of 47 IARTs were successfully ablated. During follow-up ranging from 3 to 46 months, 16 (80%) of 20 patients remained free of recurrence. Success was similar in the first 7 (group 1) and last 13 patients (group 2), but fluoroscopy time decreased from 60 ± 30 to 24 ± 9 min/procedure, probably related to the increasing experience and ability to monitor catheter position non-fluoroscopically. CONCLUSIONS Entrainment mapping combined with three-dimensional electroanatomic mapping allows delineation of complex re-entry circuits and critical isthmuses as targets for ablation. Radiofrequency catheter ablation is a reasonable option for treatment of IARTs related to repair of CHD.

Adult patients with congenital heart (ACH) disease are a product of medical and surgical advances of the second half of the twentieth century. A review at our own institution revealed that the 17-year survival of those born with all types of congenital heart disease was 84%. Despite the achievements of pediatric caregivers, many of these patients, particularly those with complex lesions, face a world not yet fully prepared for their adult care. Throughout the stages of their adolescent and adult years, congenital heart disease “survivors” present with problems often different from those encountered in their pediatric lives. Novel medical, surgical and catheter-based diagnostics and therapeutics have led to an increased use of the catheterization laboratory in the management of these patients. While simple defects predominated in the catheterization laboratory three decades ago, a marked shift toward more complex lesions was evident 20 years later 1. In the past decade however, with the advent of interventional procedures, simple lesions such as pulmonary stenosis, patent ductus arteriosus and atrial septal defect are once again being catheterized.

Grow old along with me! The best is yet to be, The last of life, for which the first was made: Our times are in His hand Who saith, “A Whole I planned, Youth shows but half; … —Robert Browning, “Rabbi Ben Ezra”1 In his poem “Rabbi Ben ( sic Ibn) Ezra,” Robert Browning acknowledges the multitude of physical and nonphysical determinants of life’s richness. His narrator sees these influences as interwoven and spanning the ages from youth through adulthood in timeless fashion. In this week’s issue of Circulation , Bouchardy and colleagues2 determine the prevalence of atrial (tachy)arrhythmias (AA) in adults with congenital heart disease (ACHD) and then analyze the prognostic implications of such. The authors’ findings carry far-reaching implications that, in a fashion that Browning’s narrator would have advocated, extend to principles, design, and application of medical care that begin at the earliest pediatric years and continue throughout the entire development and aging of the ACHD population. Article see p 1679 Nearly all physicians come into contact with ACHD because current estimates support a prevalence of >1 million adults and nearly 1 million children with congenital heart disease in the United States.3 Analyses of large administrative data sets have emphasized the growing burden (in excess to matched controls) of both cardiovascular and noncardiovascular (diabetes mellitus, renal dysfunction, obesity) disease currently existing in this ACHD population.4 Similarly, all physicians care for patients with AAs, the most common heart rhythm disorder that affects adults. Atrial fibrillation dominates (affecting >2.3 million Americans, with a prevalence of 0.4% to 5.5%, depending on age) and is a major source of morbidity and mortality because its onset heralds a 4- to 5-fold increase in stroke, a doubling of risk for dementia, a tripling of …

It has long been recognized that manifestations of congenital heart disease extend beyond the cardiovascular system. The extracardiac ramifications of congenital heart disease are multifaceted and bidirectional. The intricate interplay between congenital heart physiology, bi- or univentricular heart function, early and/or ongoing cyanosis, surgical and residual sequelae, and multisystemic effects are increasingly appreciated. Examples include the well-characterized neurological, hematologic, respiratory, skeletal, and hepatic consequences of cyanosis1; thromboembolic complications in surgically palliated univentricular hearts2; and dysregulation of the autonomic nervous system in various forms of congenital heart disease.3 Article p 2320 In the present issue of Circulation , Dimopoulos and associates4 provide original and important insights regarding the relationship between congenital heart disease and impaired renal function. They examine the prevalence of renal dysfunction across a wide-spectrum of adult congenital heart defects and explore implications on overall death rates. ### Heterogeneity of the Study Population A general and common challenge in congenital heart disease research that encompasses a broad spectrum of cardiac disorders is the complexity and marked heterogeneity of the study population. From a methodological perspective, there is often a trade-off between heterogeneity of the patient population and the precision of a given statistical inference. When the objective is to provide a global perspective on a novel association, such as renal dysfunction and death in adults with congenital heart disease, however, heterogeneity of the patient population may be advantageous. The nonrestrictive inclusion criteria enhance generalizability of the findings to a broad target population. Although the reported overall 9.3% prevalence of moderate to severe renal dysfunction must be interpreted within the context of a high-volume referral center, it reflects a wide spectrum of congenital heart disease subtypes. Heterogeneity of the study population also allows exploration of gradients …

Background —Hemodynamic efficiency of Fontan circulation is believed to be a major determinant of outcome. Prior research on flow dynamics in different modifications of Fontan circulation used in vitro models and computer-based simulation. This study was designed to compare in vivo flow dynamics in the systemic venous pathway between patients with atriopulmonary anastomosis (APA) and those with total cavopulmonary connection (TCPC). Methods and Results —Multidimensional phase-velocity magnetic resonance imaging (PV-MRI) studies were performed on 10 patients who had undergone a modified Fontan operation (5 with TCPC and 5 with APA) and were free of symptoms. The groups were comparable in terms of age and body surface area. The interval since surgery was longer for APA than for TCPC subjects. In each subject, the phase-velocity data sets were used to generate dynamic velocity-vector maps and to calculate quantitative flow indices describing the 3-dimensional blood-flow patterns throughout the cardiac cycle at the widest diameter of the Fontan pathway. Mean flow rate was comparable between groups. Velocity-vector maps showed areas of flow reversal, flow stagnation, and circular flow within APA but not TCPC pathways. Analysis of quantitative flow indices showed that compared with the APA group, flow velocities in the TCPC patients were significantly higher (mean velocity, 14±6 cm/s versus 5±3 cm/s; P =0.02), less variable (coefficient of variation, 19±2% versus 37±3.5%; P P =0.03). APA pathways were significantly more dilated than were TCPC pathways ( P R 2 =0.6, P =0.09). Fontan pathway dilatation correlated with flow velocity variability ( R 2 =0.57, P =0.01) and inversely with flow unidirectionality ( R 2 =0.75, P =0.001). Conclusions —Blood flow patterns are more organized and uniform in TCPC than in APA pathways and are significantly influenced by pathway diameter. We speculate that TCPC may result in a more hemodynamically efficient circulation than APA because of differences in pathway dimension and uniformity.

Congenital heart disease incidence has remained stable,1 but longevity has improved. There are more than 787 000 adults with congenital heart disease in the United States.2 Adults with congenital heart disease remain at risk for frequent hospitalizations.

Congenital heart disease (CHD) incidence has remained stable but longevity has improved, resulting in a growing adult CHD population. Previous reports suggested a rise in US CHD hospitalizations from 1998-2005. It is unclear if those trends have continued and whether pediatric CHD admissions have

Background Isolated peripheral pulmonary artery stenosis (PPS) in the adult is rare and frequently unsuspected. We review in this article our experience with 12 adult patients with isolated PPS, half of whom had been previously diagnosed with chronic pulmonary thromboembolic disease. Methods and Results The presentation, evolution, and management of 12 adults with isolated PPS, 17 to 51 years of age (mean, 36.2±9.7 years), were evaluated. Presenting symptoms were dyspnea and fatigue. Three patients had New York Heart Association (NYHA) functional class III or greater. Lung perfusion scans revealed multiple segmental abnormalities in flow distribution in all patients. Oxygen desaturation at rest was present in 4 patients. At catheterization, right ventricular (RV) pressure was suprasystemic in 2 patients, systemic in 1, and more than half-systemic in 7. All had multiple bilateral nonuniform stenoses in segmental and subsegmental arteries. Balloon pulmonary angioplasty (BPA) to decrease RV hypertension and improve pulmonary flow distribution was performed in 11 patients. After BPA, vessel diameter increased >50% in 10 patients, distal pulmonary artery pressure increased ≥30% in 6, and RV pressure decreased >30% in 5. One patient died shortly after BPA as a result of pulmonary hemorrhage. Immediate procedural success was achieved in 9 of 11 patients. At a mean follow-up period of 52±32 months, 7 patients had sustained symptomatic improvement (NYHA class I-II). Conclusions We describe a severe syndrome of isolated PPS in the adult that mimics chronic pulmonary thromboembolic disease. Pulmonary hemodynamics and angiography are required for definitive diagnosis. BPA may offer these patients successful short-term reduction in RV hypertension and alleviation of symptomatology.

Because the arterial switch operation has become the routine surgical approach for transposition of the great arteries, there is increasing awareness of adverse sequelae in some survivors. For the arterial switch to be considered the procedure of choice for transposition of the great arteries, long-term patency and normal function of the translocated coronary arteries must be achieved. We reviewed the cineangiograms and hemodynamic data in 366 patients who underwent postoperative catheterization after arterial switch operation. Of these, 13 patients (3%) had previously unsuspected coronary abnormalities diagnosed angiographically. No patient had noninvasive evidence of resting systolic dysfunction. Findings included left main coronary artery stenosis (n = 3) or occlusion (n = 2), anterior descending coronary artery stenosis (n = 1) or occlusion (n = 2), right coronary artery stenosis (n = 1) or occlusion (n = 1), and small coronary artery fistulas (n = 3). One patient died suddenly 3.3 years after surgery, 1 patient is lost to follow-up, and the remaining 10 patients are alive and asymptomatic up to 11 years after surgery.

Congenital heart disease (CHD) is common in patients with Down syndrome (DS). While DS survival has improved, there are limited data on morbidity, mortality and health care resource use in DS-CHD adults. We analyzed hospitalizations for patients with and without DS, 18-64 years old with CHD

Detailed imaging of pulmonary artery (PA) anatomy and significant aorto-pulmonary collaterals (APCs) is crucial for surgical planning and follow-up in patients with complex congenital heart disease (CHD) and pulmonary stenosis or atresia. Because examination by echocardiography is often technically limited and catheterization is invasive, this study evaluated the diagnostic accuracy of magnetic resonance imaging (MRI) as an alternate non-invasive tool. Thirteen patients (median age 28 years, range: 1–44 years) underwent both cardiac catheterization and MRI within a median of two months (range 0.1–8 months). Diagnoses included tetralogy of Fallot (TOF) with pulmonary atresia (n = 8), TOF with pulmonary stenosis (n = 2), single left ventricle with pulmonary stenosis (n = 2), and complex heterotaxy with pulmonary stenosis (n = 1). The MRI sequences used in this study were ECG-gated spin echo and gradient echo sequences acquired in multiple planes. Compared to catheterization, MRI had 100% sensitivity and specificity for the diagnosis of main PA (n = 6) and branch PA (n = 13) hypoplasia or stenosis, as well as discontinuous (n = 4) or absent (n = 10) branch PAs. There was complete agreement between catheterization and MRI identification of significant APCs (n = 18). Main PA atresia was noted by MRI in four patients but was not definitively seen by catheterization in any. MRI but not catheterization precisely defined the distance between discontinuous PAs and their relationship to other mediastinal structures. In conclusion, cardiac MRI is a reliable non-invasive imaging modality to define PA and APC anatomy in patients with complex pulmonary stenosis or atresia.

The use of nitric oxide as an agent to reduce pulmonary artery pressure in a pregnancy complicated by pulmonary hypertension is reported for the first time. This therapy can reduce pulmonary vascular resistance and therefore potentially enable the right ventricle to better compensate for the physiologic changes of pregnancy.

WOMEN WITH CONGENITAL HEART DISEASE DAVID W. OUYANG, PAUL KHAIRY, SUSAN FERNANDES, KATHERINE ECONOMY, AVIVA LEE-PARRITZ, MICHAEL LANDZBERG, Brigham and Women’s Hospital, Maternal Fetal Medicine, Boston, Massachusetts, Children’s Hospital, Boston, Massachusetts, Boston Medical Center, Maternal Fetal Medicine, Boston, Massachusetts OBJECTIVE: A composite risk index to predict maternal cardiac complications has been proposed as a contemporary approach to risk stratify pregnant women with heart disease. We sought to independently validate this risk index in women with congenital heart disease. STUDY DESIGN: We conducted a retrospective, single center, cohort study examining maternal outcomes in women with congenital heart disease who delivered between 1998-2004. Patients received 1 point for each of the following predictors: Previous cardiac event (h/o CHF/stroke/arrhythmia); baseline NYHA class >2 or central cyanosis; LV outflow tract obstruction; and LVEF ! 40%. The cardiac event rates were stratified by the risk index scoring system and compared to the predicted rate. The predicted rate of a primary cardiac event in pregnancies with 0, 1, and R2 points is 5%, 27% and 75% respectively. Primary events were defined as CHF; sustained arrhythmia; stroke; cardiac arrest; and cardiac death. RESULTS: 53 patients were studied who completed a total of 83 pregnancies. All patients had a baseline NYHA class %2. Six patients had a h/o CHF, eleven had a h/o arrhythmia and one patient had a h/o stroke. No patients had central cyanosis or LVEF !40%. A primary cardiac event occurred in 15 pregnancies, all of which were cases of CHF (n = 9) and arrhythmia (n = 8). In two pregnancies, both CHF and arrhythmia were observed. The predicted rate of a primary cardiac event based on the composite risk index was similar to the observed rate (Table). CONCLUSION: This study represents the largest single institution experience evaluating cardiac outcomes in pregnant women with congenital heart disease. It provides the first independent validation of the risk index scoring system proposed to predict adverse cardiac events in pregnant women with heart disease.

The natural history of patients (pts) undergoing surgical repair of ostium primum atrial septal defects (ASD-1) in adulthood remains unclear. We followed 33 pts who underwent surgical correction of ASD-l at our institution at ages 20–73 years (mean = 42), 12 of these patients were over the age of 50 at the time of surgery. Moderate preoperative exercise incapacity (NYHA Class g2) was present in 4 pts, and 6 were in atrial fibrillation. Preoperative mean pulmonary artery pressure g25 mmHg, pulmonary vascular resistance g4 Wood units, or moderate-severe mitral regurgitation was present in 8, 4, and 5 pts, respectively, Autologous pericardium was used to patch the ASD-l in 30 pts (91 %). Mitral valvuloplasty, consisting of cleft repair (n = 10) and mitral valve replacement (n = 2), were performed selectively. At a mean follow-up of 5.3 years (1 month-18.2 years), all 28 surviving pts are free of exercise limitation (NYHA Class 1). Late post-operative deaths have occurred in 5 pts (15%); related to myocardial infarction, stroke, hepatic failure, renal failure, or sepsis. Reoperation was required in 2 pts (6%); for a residual ASD-1 (n = 1). and severe mitral regurgitation (n = 1), both within the first postoperative year. The presence of advanced age at operation, symptoms, atrial arrhythmias, mitral regurgitation, or moderately increased pulmonary vascular resistance did not predict late postoperative mortality, complications or functional capacity. Conclusion ASD-1 can be repaired in adult pts with the expectation of excellent long-term results, independent of age at operation and preoperative mitral valve function; and despite the presence of atrial fibrillation, or moderately elevated pulmonary vascular resistance.

Adult patients (pts) with cyanotic congenital heart disease (CCHD) and elevation (↑) in pulmonary vascular resistance (PVR) may have a marked decrease in exercise capacity (EX) and quality of life due to intravascular volume overload, ventricular dysfunction and cyanosis-related morbidity. Concerns of increasing right-to-left shunting and cyanosis have limited characterization of the safety and effects of angiotensin converting enzyme inhibition (ACEI) in these pts. We evaluated ACEI in 7 symptomatic adults (ages 29–36 yrs) with CCHD and ↑PVR from 6/92 to 6/94. Intracardiac shunting was present at the ventricular level (single ventricle, double outlet right ventricle) in 3 pts, at the atrial septum (Ebstein's malformation, peripheral pulmonary stenoses) in 2 pts, and at both levels (AV canal defect) in 2 pts. All pts had marked exercise incapacity; 6/7 were candidates for heart-lung transplantation. Baseline serum hemoglobin (Hgb) oxygen saturation (O2sat) and 6 minute walking capacity (6 min) were compared before and after 6 weeks of ACEI. Baseline Hgb was ≥6 gm/dl (19.3 ± 1.7) and O2sat was ≤90% (79 ± 4) in all pts. ACEI caused no decrease in O2sat or increase in Hgb in any pt. When compared to baseline, EX increased ≥30% (6 min: 326 ± 129 vs 441 ± 160 yds) with ACEI in 5/5 pts (p = 0.01). All pts noted subjective functional improvement with ACEI; during treatment 3/6 pts removed themselves from consideration for organ transplantation and remain well. Our preliminary findings suggest that short-term ACEI is safe and may improve exercise capacity and quality of life in adult pts with CCHD and ↑PVR . Further investigation is required to elucidate the mechanisms of these benefits and to determine the long-term effects of ACEI in this cohort.