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Highlights •Many patients with adult CHD will experience a progressive decline in cardiopulmonary function leading to advanced HF. •These high-risk patients present unique anatomic and physiological challenges that may not be amenable to repair. •For highly selected patients, mechanical circulatory support and heart transplantation offer excellent short- and long-term outcomes. •Prospective registries are needed to determine optimal timing of referral, risk factors for adverse outcomes, and strategies for preservation of end-organ function.

[Extract] The 2018 ACHD guideline is a full revision of the “2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease” (S1.4-1), which was the first U.S. guideline to be published on the topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and growing ACHD expertise to develop recommendations. Congenital heart disease (CHD) encompasses a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac development but does not include inherited disorders that may have cardiac manifestations such as Marfan syndrome or hypertrophic cardiomyopathy. Also not included are anatomic variants such as patent foramen ovale. Valvular heart disease (VHD) may be congenital, so management overlaps with the “2014 AHA/ACC Guidelines for the Management of Patients With Valvular Heart Disease” (S1.4-2), particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the diagnosis and treatment of congenital valve disease when it differs from acquired valve disease, whether because of anatomic differences, presence of concomitant lesions, or differences to consider given the relatively young age of patients with ACHD. This guideline is not intended to apply to children (

In the contemporary era, nearly 85% of children with congenital heart disease will reach adulthood. Despite optimal medical and surgical treatment, many will experience a progressive decline in cardiopulmonary function leading to advanced heart failure. These patients present unique anatomic and physiological challenges to the care team, and unlike adults with acquired heart disease who progress to severe heart failure, advanced treatment options such as mechanical circulatory support and cardiac transplant may be limited. Severe ventricular dysfunction and/or pulmonary hypertension may not be amenable to corrective repair. Heart transplantation with or without mechanical circulatory support may be the only option for highly selected patients. The aim of this review is to describe advanced heart failure therapies for adults with congenital heart disease, including the general approach to evaluation and management, pre- and post-operative care, anticipated short- and long-term outcomes, and future directions for clinical care and research.

Congenital heart disease (CHD) is the most common birth defect, affecting about 0.8% of live births. Advances in recent decades have allowed >85% of children with CHD to survive to adulthood, creating a growing population of adults with CHD. Little information exists regarding survival, demographics, late outcomes, and comorbidities in this emerging group, and multiple barriers impede research in adult CHD. The National Heart, Lung, and Blood Institute and the Adult Congenital Heart Association convened a multidisciplinary working group to identify high-impact research questions in adult CHD. This report summarizes the meeting discussions in the broad areas of CHD-related heart failure, vascular disease, and multisystem complications. High-priority subtopics identified included heart failure in tetralogy of Fallot, mechanical circulatory support/transplantation, sudden cardiac death, vascular outcomes in coarctation of the aorta, late outcomes in single-ventricle disease, cognitive and psychiatric issues, and pregnancy.

Fixed right ventricular outflow obstruction, such as pulmonary valve stenosis, causes a systolic pressure difference between the right ventricle (RV) and pulmonary artery (PA). This is an established confounder of PA pressure estimation using tricuspid regurgitation flow velocity and must be

Endocarditis of the Melody (Medtronic, Minneapolis, MN) transcatheter pulmonary valve (TPV) has emerged as a potentially serious complication, challenging to diagnose and treat. A 53-year-old male with Tetralogy of Fallot and pulmonary atresia, right ventricle to pulmonary artery conduit in

Objectives The goal of this project was to quantify the prevalence of gaps in cardiology care, identify predictors of gaps, and assess barriers to care among adult congenital heart disease (adult CHD) patients. Background Adult CHD patients risk interruptions in care that are associated with undesired outcomes. Methods Patients (18 years of age and older) with their first presentation to an adult CHD clinic completed a survey regarding gaps in, and barriers to, care. Results Among 12 adult CHD centers, 922 subjects (54% female) were recruited. A >3-year gap in cardiology care was identified in 42%, with 8% having gaps longer than a decade. Mean age at the first gap was 19.9 years. The majority of respondents had more than high school education and knew their heart condition. The most common reasons for gaps included feeling well, being unaware that follow-up was required, and complete absence from medical care. Disease complexity was predictive of a gap in care with 59% of mild, 42% of moderate, and 26% of severe disease subjects reporting gaps (p Conclusions Adult CHD patients have gaps in cardiology care; the first lapse commonly occurred at age ∼19 years, a time when transition to adult services is contemplated. Gaps were more common among subjects with mild and moderate diagnoses and at particular locations. These results provide a framework for developing strategies to decrease gaps and address barriers to care in the adult CHD population.

Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva is increasingly recognized by cardiac imaging. Although most AAOCA subtypes are benign, autopsy studies report an associated risk of sudden death with interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA). Despite efforts to identify high-risk ALCA and ARCA patients who may benefit from surgical repair, debate remains regarding their classification, prevalence, risk stratification, and management. We comprehensively reviewed 77 studies reporting the prevalence of AAOCA among >1 million patients, and 20 studies examining outcomes of interarterial ALCA/ARCA patients. Observational data suggests that interarterial ALCA is rare (weighted prevalence = 0.03%; 95% confidence interval [CI]: 0.01% to 0.04%) compared with interarterial ARCA (weighted prevalence = 0.23%; 95% CI: 0.17% to 0.31%). Recognizing the challenges in managing these patients, we review cardiac tests used to examine AAOCA and knowledge gaps in management.

Objectives This study sought to elucidate referral patterns and barriers to adult congenital heart disease (ACHD) care, as perceived by pediatric cardiologists (PCs). Background Management guidelines recommend that care of adults with moderate/complex congenital heart disease be guided by clinicians trained in ACHD. Methods A cross-sectional survey was distributed to randomly selected U.S. PCs. Results Overall response rate was 48% (291 of 610); 88% (257 of 291) of respondents met inclusion criteria (outpatient care to patients >11 years of age). Participants were in practice for 18.2 ± 10.7 years; 70% were male, and 72% were affiliated with an academic institution; 79% stated that they provide care to adults (>18 years). The most commonly perceived patient characteristic prompting referral to ACHD care was adult comorbidities (83%). The most perceived barrier to ACHD care was emotional attachment of parents and patients to the PC (87% and 86%, respectively). Clinician attachment to the patient/family was indicated as a barrier by 70% of PCs and was more commonly identified by responders with an academic institutional affiliation (p = 0.001). A lack of qualified ACHD care providers was noted by 76% of PCs. Those affiliated with an academic institution were less likely to identify this barrier to ACHD care (p = 0.002). Conclusions Most PC respondents in the United States provide care to ACHD patients. Common triggers that prompt referral and perceived barriers to ACHD care were identified. These findings might assist ACHD programs in developing strategies to identify and retain patients, improve collaborative care, and address emotional needs during the transition and transfer process.

The Adult Congenital and Pediatric Cardiology (AC/PC) Section was established to develop a clear voice within the American College of Cardiology and address the myriad issues facing the congenital heart disease profession. The Section is governed by the AC/PC Council, which includes pediatric cardiologists, adult congenital cardiologists, a cardiac care associate, and a fellow-in-training member. The Council is responsible for bidirectional communication between the College's Board of Trustees and the AC/PC Section members. Since its founding in 2004, Section objectives have been defined by the College's mission: to advocate for quality cardiovascular care through education, research promotion, and the development and application of standards and guidelines and to influence health care policy. The pillars of the College—advocacy, quality, education, and member engagement—serve as the defining template for the Section's strategy. The Section has developed work groups in advocacy, clinical practice, education and training, quality, and publications. A separate leadership group has been developed for adult congenital heart disease. Work groups are open to all Section members. Recognition of the importance of lifelong care in congenital heart disease led Section leaders to incorporate pediatric cardiology and adult congenital heart disease content into each of the work groups. There are more than 1,200 Section members, with nearly 400 members actively contributing to Section activities. This article outlines Section efforts to date and highlights significant successes to date.

“I was so much older then, I’m younger than that now” —Bob Dylan [(1)][1] Across the years, standardization of care for children and adults with congenital heart disease has paled in comparison with that witnessed for acquired heart disease. Congenital cardiology’s experience with

It is important that the medical profession play a central role in critically evaluating the use of diagnostic procedures and therapies introduced and tested for detection, management, or prevention of disease. Rigorous, expert analysis of the available data documenting absolute and relative

Congenital heart disease (CHD) is the most common birth defect, affecting about 0.8% of live births. Advances in recent decades have allowed >85% of children with CHD to survive to adulthood, creating a growing population of adults with CHD. Little information exists regarding survival, demographics, late outcomes, and comorbidities in this emerging group, and multiple barriers impede research in adult CHD (ACHD). The National Heart, Lung, and Blood Institute and the Adult Congenital Heart Association convened a multidisciplinary Working Group to identify high-impact research questions in ACHD. This report summarizes the meeting discussions in the broad areas of CHD-related heart failure, vascular disease and multisystem complications. High-priority subtopics identified included heart failure in tetralogy of Fallot, mechanical circulatory support/transplantation, sudden cardiac death, vascular outcomes in coarctation of the aorta, late outcomes in single ventricle disease, cognitive and psychiatric issues, and pregnancy.

Transcatheter therapies in structural heart disease have evolved tremendously over the past 15 years. Since the introduction of the first balloon-expandable valves for stenotic lesions with implantation in the pulmonic position in 2000, treatment for valvular heart disease in the outflow position has become more refined, with newer-generation devices, alternative techniques, and novel access approaches. Recent efforts into the inflow position and regurgitant lesions, with transcatheter repair and replacement technologies, have expanded our potential to treat a broader, more heterogeneous patient population. The evolution of multimodality imaging has paralleled these developments. Three- and 4-dimensional visualization and concomitant use of novel technologies, such as fusion imaging, have supported technical growth, from pre-procedural planning and intraprocedural guidance, to assessment of acute results and follow-up. A multimodality approach has allowed operators to overcome many limitations of each modality and facilitated integration of a multidisciplinary team for treatment of this complex patient population.

The Working Group on research in adult congenital heart disease (ACHD) was convened in September 2004 under the sponsorship of National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases, National Institutes of Health, Department of Health and Human Services, to make recommendations on research needs. The purpose of the Working Group was to advise the NHLBI on the current state of the science in ACHD and barriers to optimal clinical care, and to make specific recommendations for overcoming those barriers. The members of the Working Group were chosen to provide expert input on a broad range of research issues from both scientific and lay perspectives. The Working Group reviewed data on the epidemiology of ACHD, long-term outcomes of complex cardiovascular malformations, issues in assessing morphology and function with current imaging techniques, surgical and catheter-based interventions, management of related conditions including pregnancy and arrhythmias, quality of life, and informatics. After research and training barriers were discussed, the Working Group recommended outreach and educational programs for adults with congenital heart disease, a network of specialized adult congenital heart disease regional centers, technology development to support advances in imaging and modeling of abnormal structure and function, and a consensus on appropriate training for physicians to provide care for adults with congenital heart disease.

The term “structural heart disease” entered the adult cardiology lexicon in 1999 and currently encompasses the base of knowledge and competencies surrounding noncoronary cardiac procedures such as transcatheter aortic valve replacement (TAVR), percutaneous mitral repair, and left atrial

A total of 190 abstracts were submitted to the Pediatric Cardiology and Adult Congenital Heart Disease Sessions; 69 were accepted for presentation either as oral or poster presentations. Three abstracts were selected for featured poster presentations in the Pediatric Cardiology/Adult Congenital

Objectives. We sought to determine the long-term survival rates and defect-related morbidity of adult patients who undergo surgical repair of an ostium primum atrial septal defect. Background. The natural history of patients undergoing such surgical repair in adulthood remains unclear. Methods. We followed up 33 patients who underwent surgical correction of an ostium primum atrial septal defect at our institution at a mean age of 42 years (range 20 to 73); 12 of these patients were >50 years old at the time of operation. Four patients had moderate preoperative exercise incapacity (New York Heart Association functional class >11) and six had atrial fibrillation. Nine and four patients, respectively, had a preoperative mean pulmonary artery pressure >25 mm Hg or pulmonary vascular resistance >4 Wood U. Autologous pericardium was used to patch the defect in 30 patients (91%). Mitral valvuloplasty, consisting of cleft repair (n=10), and mitral valve replacement (n = 2) were performed selectively. Results. There were no operative deaths. At a mean follow-up interval of 5.3 years (range 1 to 18.2), all 28 surviving patients are free of exercise limitation (functional class 1). Late postoperative deaths occurred in five patients (15%) and were related to myocardial infarction, stroke, hepatic failure, renal failure or sepsis. Reoperation within the 1st postoperative year was required in two patients (6%) because of a residual ostium primum defect in one and severe mitral regurgitation in the other. 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. Conclusions. An ostium primum defect can be repaired in adult patients 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.

Objectives. We investigated the effect of inhaled nitric oxide and infused acetylcholine in patients with pulmonary hypertension undergoing cardiac catheterization before cardiopulmonary transplantation.Background. The fate of patients under consideration for transplantation of the heart or lungs, or both is influenced by the evaluation of their pulmonary vascular reactivity.Methods. We evaluated 11 patients who were classified into two groups on the basis of mean left atrial pressure >15 mm Hg (group I, n = 6) or ≤15 mm Hg (group II, n = 5). All patients inhaled nitric oxide at 80 ppm. This was preceded by an infusion of 10−6mol/liter of acetylcholine in seven consecutive patients (n = 3 in group I, n = 4 in group II).Results. In group I, inhaled nitric oxide decreased pulmonary artery pressure from (mean ± SE) 71 ± 13 to 59 ± 10 mm Hg (p < 0.05), pulmonary vascular resistance from 14.9 ± 3.8 to 7.6 ± 1.7 Um2(p < 0.05) and intrapulmonary shunt fraction from 17.8 ± 3.6% to 12.7 ± 2.1% (p < 0.05). Left atrial pressure tended to increase from 27 ± 4 to 32 ± 5 mm Hg (p = 0.07). In group II pulmonary vascular resistance decreased in response to nitric oxide from 36.4 ± 9.0 to 31.1 ± 7.9 Um2(p < 0.05). Cardiac index, systemic pressure and resistance did not change in either group. Seven patients who received acetylcholine had no significant alteration in pulmonary hemodynamic variables.Conclusions. These preliminary observations suggest that nitric oxide is a potent pulmonary vasodilator with minimal systemic effects. It may be useful in discriminating patients needing combined heart and lung transplantation from those requiring exchange of the heart alone.

While exercise echocardiography is often used as a noninvasive screening strategy to diagnose pulmonary hypertension, it is technically challenging and limited data exist regarding the accuracy of echo estimates during exercise. Among 62 patients with unexplained exertional intolerance undergoing

Echocardiography is often used to estimate pulmonary arterial pressure (PAP) at rest and with exercise; it is well known that such estimates must account for fixed right ventricular outflow tract (RVOT) obstruction. Dynamic RVOT obstruction during exercise could create a similar issue and thereby

Cardiac rehabilitation (CR) programs improve exercise capacity and quality of life while reducing mortality in adults with acquired heart disease. These programs have not been extensively studied in adults with congenital heart disease (CHD). We performed a prospective, randomized, controlled trial

This study sought to elucidate referral patterns and barriers to adult congenital heart disease (ACHD) care, as perceived by pediatric cardiologists (PCs).Management guidelines recommend that care of adults with moderate/complex congenital heart disease be guided by clinicians trained in ACHD.A cross-sectional survey was distributed to randomly selected U.S. PCs.Overall response rate was 48% (291 of 610); 88% (257 of 291) of respondents met inclusion criteria (outpatient care to patients11 years of age). Participants were in practice for 18.2 ± 10.7 years; 70% were male, and 72% were affiliated with an academic institution; 79% stated that they provide care to adults (18 years). The most commonly perceived patient characteristic prompting referral to ACHD care was adult comorbidities (83%). The most perceived barrier to ACHD care was emotional attachment of parents and patients to the PC (87% and 86%, respectively). Clinician attachment to the patient/family was indicated as a barrier by 70% of PCs and was more commonly identified by responders with an academic institutional affiliation (p = 0.001). A lack of qualified ACHD care providers was noted by 76% of PCs. Those affiliated with an academic institution were less likely to identify this barrier to ACHD care (p = 0.002).Most PC respondents in the United States provide care to ACHD patients. Common triggers that prompt referral and perceived barriers to ACHD care were identified. These findings might assist ACHD programs in developing strategies to identify and retain patients, improve collaborative care, and address emotional needs during the transition and transfer process.

We read with interest and appreciation 3 recent papers in the Journal on the bicuspid aortic valve (BAV). Fernandez et al. ([1][1]) describe distinct developmental patterns for mice and hamsters with right-noncoronary and right-left coronary cusp fusion, respectively. Incredibly, William Osler

ObjectivesThe objective of this study was to document the prevalence of chronic venous insufficiency (CVI) and its associated factors in adults with Fontan physiology.BackgroundAs the population of adults with complex congenital heart disease and Fontan physiology increases, so does the occurrence of highly morbid and mortal outcomes, including heart failure and thromboembolism. The presence of abnormal peripheral hemodynamic conditions in this population and their potential contribution to adverse outcomes is not well known. The primary objective of this study was to document the prevalence of CVI in adults with Fontan physiology.MethodsA total of 159 adults with Fontan physiology from 7 adult congenital heart centers were prospectively assessed for lower extremity CVI, with the assignment of clinical, etiological, anatomical, and pathophysiological classification grades, and compared with age-matched and sex-matched controls. Leg photographs were independently reassessed to confirm interobserver reliability.ResultsThe prevalence of CVI was significantly greater in the Fontan population (60%; 95% confidence interval [CI]: 52% to 68%) compared with healthy controls (32%; 95% CI: 15% to 54%) (p = 0.008). Strikingly, the prevalence of severe CVI (clinical, etiological, anatomical, and pathophysiological grade ≥4) was significantly higher in the Fontan group (22%; 95% CI: 16% to 29%) versus the healthy controls (0%; 95% CI: 0% to 14%) (p = 0.005). In a multivariate analysis, several factors were independently associated with severe CVI, including increased numbers of catheterizations with groin venous access, lower extremity itching, and deep venous thrombosis.ConclusionsCVI is common in adult patients with congenital heart disease with Fontan physiology. The contribution of abnormal peripheral hemodynamic conditions to comorbidities, including thromboembolism and heart failure, and interventions to improve peripheral hemodynamic conditions require further exploration.

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

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

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.

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

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

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.

In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension [PAH]) were proposed. The main change was to withdraw persistent pulmonary hypertension of the newborn (YPPH) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1". Pulmonary hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric pulmonary hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental pulmonary hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4. (J Am Coll Cardiol 2013;62:D34-41) a 2013 by the American College of Cardiology Foundation.