Long-Term Outcomes of Unrepaired Isolated Partial Anomalous Pulmonary Venous Connection With an Intact Atrial Septum.

The natural history of an unrepaired isolated partial anomalous pulmonary venous connection(s) (PAPVC) and the absence of other congenital anomalies remains unclear. This study aimed to expand the understanding of the clinical outcomes in this population. Isolated PAPVC with an intact atrial septum is a relatively uncommon condition. There is the perception that patients with isolated PAPVC are usually asymptomatic, that the lesion generally has a limited hemodynamic impact, and that surgical repair is rarely justified. For this retrospective study, we reviewed our institutional database to identify patients with either 1 or 2 anomalous pulmonary veins that drain a portion of but not the complete ipsilateral lung. Patients with previous surgical cardiac repair, coexistence of other congenital cardiac anomalies that would result in either pretricuspid or post-tricuspid loading of the right ventricle (RV), or scimitar syndrome were excluded. We reviewed their clinical course over the follow-up period. We identified 53 patients; 41 with a single and 12 with 2 anomalous PAPVC. A total of 30 patients (57%) were men, with a mean age at the latest clinic visit of 47 ± 19 years (18 to 84 years). Turner syndrome (6 of 53, 11.3%), bicuspid aortic valve (6 of 53, 11.3%), and coarctation of the aorta (5 of 53, 9.4%) were commonly associated anomalies. A single anomalous left upper lobe vein was the most commonly identified variation. More than half of the patients were asymptomatic. Cardiopulmonary exercise test demonstrated a maximal oxygen consumption of 73 ± 20% expected (36 to 120). Transthoracic echocardiography demonstrated a mean RV basal diameter of 4.4 ± 0.8 cm, RV systolic pressure of 38 ± 13 (16 to 84) mm Hg. A total of 8 patients (14.8%) had ≥moderate tricuspid regurgitation. Cardiac magnetic resonance in 42 patients demonstrated a mean RV end-diastolic volume index of 122 ±3 0 ml/m2 (66 to 188 ml/m2), of which in 8 (14.8%), it was >150 ml/m2. Magnetic resonance imaging-based Qp:Qs was 1.6 ± 0.3. A total of 5 patients (9.3%) had established pulmonary hypertension (mean pulmonary artery pressure ≥25 mm Hg). In conclusion, isolated single or dual anomalous pulmonary venous connection is not necessarily a benign congenital anomaly because a proportion of patients develop pulmonary hypertension and/or RV dilation. Regular follow-up and on-going patient surveillance with cardiac imaging is advised.

Cardiac magnetic resonance imaging characteristics and pregnancy outcomes in women with Mustard palliation for complete transposition of the great arteries.

BACKGROUND: Women with transposition of the great arteries (TGA) following atrial redirection surgery are at risk of pregnancy-associated arrhythmia and heart failure. The cardiovascular magnetic resonance imaging (CMR) characteristics of these women and the relationship of CMR findings to pregnancy outcomes have not been described. METHODS: We included 17 women with atrial redirection surgery and CMR within 2 years of delivery. RESULTS: All women were asymptomatic at baseline (New York Heart Association Class 1). CMR studies were completed pre-pregnancy in 3, antepartum/peripartum in 2, and postpartum in 12 women. Three women (3/17, 18%) experienced major cardiovascular events related to pregnancy: cardiac arrest (n = 1) and symptomatic atrial arrhythmia (n = 2). Median gestational age at delivery was 38 weeks (24-39 weeks) and birth weight was 2770 g (2195-3720 g). Complications were seen in 3 offspring (3/17, 18%): death (n = 1) and prematurity (n = 2). CMR characteristics included median right ventricular end diastolic volume 119 mL/m2 (range 85-214 mL/m2) and median right ventricular ejection fraction (RVEF) 37% (range 30-51%). All women with cardiovascular complications had an RVEF < 35% (range 32-34%). The association between RVEF < 35% and cardiovascular complications trended towards statistical significance (p = 0.05). No statistically significant differences in CMR measurements were found between those with and without neonatal complications. CONCLUSIONS: While the majority of women in this cohort had successful outcomes following pregnancy, important cardiovascular complications were seen in a significant minority, all of whom had an RVEF < 35%. The preliminary findings of our study provide impetus for a larger prospective study to evaluate the prognostic role of CMR in pregnant women with atrial redirection surgery.

Outcomes of Aortic Valve-Sparing Operations in Marfan Syndrome.

BACKGROUND: In many cardiac units, aortic valve-sparing operations have become the preferred surgical procedure to treat aortic root aneurysm in patients with Marfan syndrome, based on relatively short-term outcomes. OBJECTIVES: This study examined the long-term outcomes of aortic valve-sparing operations in patients with Marfan syndrome. METHODS: All patients with Marfan syndrome operated on for aortic root aneurysm from 1988 through 2012 were followed prospectively for a median of 10 years. Follow-up was 100% complete. Time-to-event analyses were calculated using the Kaplan-Meier method with log-rank test for comparisons. RESULTS: A total of 146 patients with Marfan syndrome had aortic valve-sparing operations. Reimplantation of the aortic valve was performed in 121 and remodeling of the aortic root was performed in 25 patients. Mean age was 35.7 ± 11.4 years and two-thirds were men. Nine patients had acute, 2 had chronic type A, and 3 had chronic type B aortic dissections before surgery. There were 1 operative and 6 late deaths, 5 caused by complications of dissections. Mortality rate at 15 years was 6.8 ± 2.9%, higher than the general population matched for age and sex. Five patients required reoperation on the aortic valve: 2 for endocarditis and 3 for aortic insufficiency. Three patients developed severe, 4 moderate, and 3 mild-to-moderate aortic insufficiency. Rate of aortic insufficiency at 15 years was 7.9 ± 3.3%, lower after reimplantation than remodeling. Nine patients developed new distal aortic dissections during follow-up. Rate of dissection at 15 years was 16.5 ± 3.4%. CONCLUSIONS: Aortic valve-sparing operations in patients with Marfan syndrome were associated with low rates of valve-related complications in long-term follow-up. Residual and new aortic dissections were the leading cause of death.

Increasing mortality burden among adults with complex congenital heart disease.

BACKGROUND: Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease. METHODS: Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029. RESULTS: Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot. CONCLUSIONS: Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease.

Facilitators of and barriers to advance care planning in adult congenital heart disease.

BACKGROUND: Most adults with congenital heart disease (CHD) are interested in discussing matters related to advance care planning (ACP) early in the disease course, yet few such conversations actually occur. We aimed to evaluate factors that impact these discussions between patients and adult CHD providers. METHODS: Two hundred adult CHD outpatients completed a survey that included factors that might impact ACP discussions with their doctors. In parallel, forty-eight providers within the Canadian Adult Congenital Heart Network completed a similar online survey. Responses were compared between the groups. RESULTS: Most providers (85%) worried that they were unable to reliably estimate life expectancy and believed that patients were not ready for end-of-life discussions if their estimated life expectancies were beyond 5 years (63%) or beyond 10 years (79%). In contrast, only 24% of patients, independent of disease complexity, thought they were not ready to talk about ACP. Most providers (83%) reported that greater certainty about patients' prognoses would help them discuss ACP. Patients thought that such discussions were best facilitated when they had trust in their doctors (85%) and believed their doctors are good at taking care of patients with CHD (78%). CONCLUSION: Despite the fact that challenges to prognostication exist, discussions about ACP should not be reserved for patients with a severely reduced life expectancy. Most patients want these discussions regardless of the complexity of their disease. The trusting and close patient-doctor relationship in adult CHD, often evolving over many years, may provide an excellent platform from which to initiate such discussions.

Maternal atrial arrhythmia detected by uterine artery Doppler.

BACKGROUND: Uterine artery Doppler ultrasound assessment is used to assess uteroplacental blood flow and the risk of placental ischemic-thrombotic injury in high-risk pregnancies. We report here a case in which this examination identified the presence of a maternal atrial arrhythmia. CASE: A 50-year-old woman with no significant past medical history received antenatal care in our fetal medicine unit clinic because of advanced maternal age, a history of recurrent pregnancy loss, multiple uterine fibroids, and a history of in vitro fertilization using donated oocytes. Uterine artery Doppler ultrasound was normal at 19 weeks, but was repeated at 33 weeks because of abnormal placental texture and maternal risks, revealing an irregular maternal heart rhythm. Paroxysmal atrial fibrillation was confirmed by Holter monitor at 34 weeks. The patient was asymptomatic and declined the cardiologist's recommendation of antepartum and postpartum anticoagulation. She remained asymptomatic and had delivery by Caesarean section at term. CONCLUSION: Uterine artery Doppler flow studies may identify a significant maternal cardiac arrhythmia.

Pulmonary valve replacement in adults with repaired tetralogy of Fallot.

There is a growing population of young adults with tetralogy of Fallot. Although surgical approaches have evolved, many adults with repaired tetralogy of Fallot have been left with residual pulmonary regurgitation. Pulmonary regurgitation is an important contributor to a number of late complications including exercise limitations, right heart failure, arrhythmia, and sudden death. Because bioprosthetic valves are used in this population, clinicians must weigh the beneficial effects of pulmonary valve replacement against the associated risks, including subsequent re-operation. In this review, we will appraise the evidence supporting pulmonary valve replacement in the adult with repaired tetralogy of Fallot, as well as the optimal timing and mode of intervention.

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: executive summary.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. They have distinctive forms of heart failure, and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death.Medical aspects that need to be considered relate to the long-term and multisystemic effects of single-ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the understanding of the late outcomes, genetics, medical therapy and interventional approaches in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. The present executive summary is a brief overview of the new guidelines and includes the recommendations for interventions. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology, including sections on genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy and contraception risks, and follow-up requirements. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome.

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part II of the guidelines includes recommendations for the care of patients with left ventricular outflow tract obstruction and bicuspid aortic valve disease, coarctation of the aorta, right ventricular outflow tract obstruction, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

Long-term results of aortic valve-sparing operations in patients with Marfan syndrome.

OBJECTIVE: The appropriateness of aortic valve-sparing operations in patients with Marfan syndrome has been questioned. This study examines the long-term results of these operations in patients with Marfan syndrome. METHODS: From 1988 to 2006, 103 consecutive patients with Marfan syndrome (mean age, 37 +/- 12 years) and aortic root aneurysm had aortic valve-sparing operations. Emergency surgery was performed in 11 patients: 8 for acute type A aortic dissection and 3 for unexplained persistent chest pain. Fourteen patients also had mitral valve surgery. The technique of aortic valve reimplantation was used in 77 patients, and aortic root remodeling was used in 26 patients. Patients were followed prospectively and underwent annual echocardiographic studies. The mean follow-up was 7.3 +/- 4.2 years and 100% complete. RESULTS: There was 1 operative death and 5 late deaths. Four of the 6 deaths were due to complications of aortic dissections. The patients' survival at 15 years was 87.2% compared with 95.6% for the general population of Ontario matched for age and sex. Seven patients had important aortic insufficiency: 4 mild to moderate, 2 moderate, and 1 moderate to severe. Freedom from greater than mild aortic insufficiency at 15 years was 79.2%. Three patients, all after aortic root remodeling, had aortic valve replacement, 2 for aortic insufficiency and 1 for endocarditis. At the most recent follow-up, 97 patients were alive: 86 were in functional class I, and 11 were in functional class II. CONCLUSIONS: Aortic valve-sparing operations provided excellent clinical outcomes in this series of patients with Marfan syndrome. Postoperatively, complications of aortic dissections were the leading cause of death.

Adults with congenital heart disease: psychological needs and treatment preferences.

OBJECTIVE: Approximately one-third of adult congenital heart disease (ACHD) patients have mood or anxiety disorders, the majority of which go untreated. The extent to which this group of patients is interested in psychological services is, however, unknown. This study investigated the perceived psychological needs of patients and their mental health treatment preferences. DESIGN: Participants completed an anonymous survey with items regarding mental health treatment history, preferences for future treatment, Internet use, and interest in peer support. RESULTS: A total of 155 ACHD patients completed study questionnaires (mean age = 39 years; 50% female). Forty percent of patients reported previous mental health treatment. Some 51% of patients (73/142) indicated significant interest in at least 1 of 7 defined areas of psychological treatment (managing mood and/or anxiety, coping with a cardiac condition, stress management, anger management, relationship difficulties, substance use, and smoking cessation). Patients were most interested in stress management and coping with heart disease; one-third of patients (48/141 and 47/141, respectively) expressed high interest in these 2 topics. There was a consistent preference for psychological treatment to be provided over the Internet vs. in person or over the telephone; 94% of the patients had Internet access. In total, 35% of patients (51/145) were interested in receiving peer support. CONCLUSIONS: It is not only health providers who recognize the importance of psychological care for ACHD patients. As a group, patients are also interested in psychological treatment and peer support. Programs are encouraged to provide psychological services and maximize opportunities for interactions with other patients.

Aortic valve preservation in patients with aortic root aneurysm: results of the reimplantation technique.

BACKGROUND: A study was conducted to determine the long-term results of aortic valve reimplantation to treat aortic root aneurysm. METHODS: Prospective follow-up with clinical assessments and echocardiography was done of 167 consecutive patients who had reimplantation of the aortic valve as treatment of aortic root aneurysm. Their mean age was 45 +/- 15 years, 78% were men, 38% had Marfan syndrome, 14% had aortic dissection, and 7% had bicuspid aortic valve. The aortic valve was reimplanted into a straight Dacron (Dupont, Wilmington, DE) tube in 89 patients and in a Dacron tube with creation of neoaortic sinuses in 78. Aortic cusp repair was performed in 66 patients, and the free margin was reinforced with a fine Gore-Tex suture (W.L. Gore & Assoc, Flagstaff, AZ) in 36. The mean follow-up was 5.1 +/- 3.8 years and was 100% complete. RESULTS: There were two operative and six late deaths. Survival at 10 years was 92% +/- 3%. Moderate aortic insufficiency developed in 3 patients, and severe developed in 2. Freedom from moderate or severe aortic insufficiency was 94% +/- 4% at 10 years. Two patients required aortic valve replacement. Freedom from aortic valve replacement was 95% +/- 4% at 10 years. At the latest follow-up, 90% of the patients were in New York Heart Association functional class I and 10% were in class II. CONCLUSIONS: Reimplantation of the aortic valve to treat patients with aortic root aneurysm is associated with excellent long-term survival and low rates of valve-related complications. Reimplantation of the aortic valve is a durable type of aortic valve repair.

Long-term results of aortic valve-sparing operations for aortic root aneurysm.

OBJECTIVES: To examine the results of aortic valve sparing for aortic root aneurysm. METHODS: Two hundred twenty consecutive patients who had aortic valve sparing for aortic root aneurysm were prospectively studied with annual clinical assessments and echocardiography. Their mean age was 46 +/- 15 years, 40% had Marfan syndrome, 17% had aortic dissection, and 7% had bicuspid aortic valve. Reimplantation of the aortic valve was performed in 167 patients and remodeling of the aortic root in 53. Aortic cusp repair was performed in 80 patients, and reinforcement of the free margin of one of the cusps with a fine polytetrafluoroethylene (Gore-Tex) suture in 48. The mean follow-up was 5.2 +/- 3.7 years and it was complete. RESULTS: There were 3 operative and 13 late deaths. Patients' survival at 10 years was 88% +/- 3%. Age older than 65 years, advanced functional class, and ejection fraction less than 40% were independent predictors of death. Moderate aortic insufficiency developed in 7 patients and severe insufficiency in 6. Freedom from moderate or severe aortic insufficiency at 10 years was 85% +/- 5% for all patients, but it was 94% +/- 4% after reimplantation and 75% +/- 10% after remodeling (P = .04). Five patients required aortic valve replacement; the freedom from valve replacement at 10 years was 95% +/- 3%. One case of endocarditis developed 11 years postoperatively. At the latest follow-up, 88% of the patients were in functional class I, and 10% were in class II. CONCLUSIONS: Aortic valve-sparing operation is associated with low rates of valve-related complications. The probability of late aortic insufficiency was lower after the reimplantation procedure than after remodeling in our experience.

Outcomes after late reoperation in patients with repaired tetralogy of Fallot: the impact of arrhythmia and arrhythmia surgery.

BACKGROUND: We evaluated outcomes in patients requiring late reoperation after tetralogy of Fallot (ToF) repair to identify risk factors for arrhythmia and determine whether arrhythmia surgery decreased the risk of subsequent death or recurrent arrhythmia. METHODS: Review was performed of all ToF patients from 1969 to 2005 undergoing reoperation late (> 1 year) after repair. Patients with associated lesions, except pulmonary atresia, were included. A total of 249 patients had 278 reoperations. Procedures at initial reoperation included pulmonary valve replacement (PVR) in 217, ablation in 63, and tricuspid valve repair/replacement in 46. Pre-reoperative arrhythmias were present in 75, including supraventricular tachycardia (SVT) in 31, ventricular tachycardia (VT) in 34, and SVT+VT in 10 patients. RESULTS: Median age at reoperation was 23 years (range, 1 to 63). Ten-year survival after reoperation was 93%, and was independent of arrhythmia status (p = 0.86). Arrhythmia patients were characterized by older age at initial repair and at late reoperation, tricuspid and pulmonary regurgitation, and longer QRS duration (p < 0.001 for all). Risk factors for post-reoperative recurrent arrhythmia were longer QRS duration and not having PVR. Longer QRS duration, with a cut-point of more than 160 msec, was associated with recurrent SVT (p = 0.004). Supraventricular tachycardia ablation improved arrhythmia-free survival (75% versus 33%, p < 0.001) but VT ablation did not (96% versus 95%, p = 0.50). However, recurrent VT occurred in only 3 patients (10%). CONCLUSIONS: Late mortality in patients undergoing reoperation after ToF repair is not impacted by pre-reoperative arrhythmia. Prolongation of QRS identifies patients at risk for recurrent VT and SVT, but recurrent VT is uncommon. Early PVR, and surgical ablation in patients with SVT, decreases arrhythmic risk.

Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair.

The timing of pulmonary valve replacement in adult patients with repaired tetralogy of Fallot remains controversial. A magnetic resonance imaging study in 17 adult patients with repaired tetralogy of Fallot reveals a statistically significant decrease in right ventricular (RV) volume (RV end-diastolic volume 163 +/- 34 to 107 +/- 26 ml/m2, p <0.001; RV end-systolic volume 109 +/- 27 to 69 +/- 22 ml/m2, p <0.001) at a mean follow-up of 21 months after pulmonary valve replacement; whereas RV systolic function remained unchanged (mean RV ejection fraction 32 +/- 7% to 34 +/- 10%, p = 0.12). In no patients with a RV end-diastolic volume >170 ml/m2 or a RV end-systolic volume >85 ml/m2 before pulmonary valve replacement were RV volumes "normalized" after surgery.