Vejlstrup N, Sørensen K, Mattsson E, Thilén U, Kvidal P, Johansson B, Iversen K, Søndergaard L, Dellborg M, Eriksson P.
Circulation. 2015 Aug 25;132(8):633-8. doi: 10.1161/CIRCULATIONAHA.114.010770. Epub 2015 Jul 16.
Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: The group from Denmark and Sweden continues to impress with this excellent long-term follow up data on the largest study of survival after the atrial switch operation for transposition of the great arteries. The atrial baffle operation, for transposition of the great arteries was introduced in the 1950’s, and continued to be in use until the arterial switch operation became the standard of care in the early 1990’s. This interesting cohort of patients is now squarely in the adult age range. They were able to identify 468 patients and of those there was a 20% 30-day mortality, and 60% were still alive after 30 years of follow-up. Most notably sinus nodal dysfunction, which necessitates pacemaker placement, is associated with an increase in mortality. Long-term atrial baffle survivors provide important insight into the outcomes of systemic morphologic right ventricle patients. All patients who underwent atrial baffle from 1967-2003 were identified, 6 total centers. Patients who required a Rastelli, were a DORV, or univentricular heart were excluded. Pacemaker was required in 15% of patients at 30 years. 39% of the patients died or underwent a heart transplant. The incidence of TGA is 312 per million which extrapolates to 1900 patients during the study period. Only 468 patients had an atrial baffle procedure, and a very small number had ASO in the late 1990’s. A less-than-perfect atrial baffle does not inherently damage the RV but may lead to baffle revision or reconstruction. The authors made it quite clear that several factors normally considered important such as (operation type [Mustard versus Senning], age at operation, institution where the operation was performed, operation early or late in the era, associated defects such as VSD and LVOTO) are not statistically significantly associated with long-term survival. It is excellent to appreciate the good long-term survival curves in our aging Mustard-Senning population.
Take home points:
- Perioperative factors during atrial switch do not influence long-term survival.
- Implantation of a pacemaker, which was required in 15% over 30 years, was the only factor identified to have an adverse effect on long-term mortality.
Thompson JL, Kuklina EV, Bateman BT, Callaghan WM, James AH, Grotegut CA.
Obstet Gynecol. 2015 Aug;126(2):346-54. doi: 10.1097/AOG.0000000000000973.
Select item 26008762
Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: This trial was designed to determine whether or not women with congenital heart disease are more likely to have medical or obstetric complications. From 2000 to 2010 there was a significant linear increase in the prevalence of CHD to 9.0 per 10,000 delivery hospitalizations. A composite negative cardiovascular outcome is more likely to occur among delivery hospitalizations with maternal CHD than in women without. The number of delivery hospitalizations with maternal CHD in the US is increasing, and it remains unclear whether the correction of the lesion affected the outcomes. Each of these hospitalizations has a high burden of medical and obstetric complications. In 2005 is was estimated that there were 1 million adults living in the US with CHD, which continues to increase at a rate of 5% per year, which leads us to more women with CHD surviving to childbearing age. White race is more prevalent among women with CHD delivery hospitalizations. Hospitalizations with CHD had a trend towards a longer hospital stay. The most commonly coded congenital heart lesions were ASD, VSD, and left-sided congenital valvular lesions. 15% of delivery hospitalizations with CHD had a nonspecific code for CHD. There were no documented deliveries documented with HLHS. Delivery hospitalizations with CHD represented 0.085% of all deliveries from 2008 to 2010, but represent 2.0% of all in-hospital deaths. In-hospital mortality, medical cardiovascular complications including myocardial infarction, cardiac arrest, arrhythmia, heart failure, and stroke as well as obstetric complications are more likely to take place among delivery hospitalizations with CHD in comparison with delivery hospitalizations without CHD. It is unfortunate that the true incidence of maternal death defined as deaths from pregnancy complications occurring during pregnancy or within 42 days of delivery remains largely unknown. Although this data is not new, or a departure from previously published data including the ROPAC data it is simply further evidence of the way in which we should be counseling our CHD patients regarding pregnancy risks during prenatal visits.
Take home points:
- There is a significant linear increase in the prevalence of deliveries complicated by CHD in the US.
- It is unfortunate that the true incidence of maternal death defined as deaths from pregnancy complications occurring during pregnancy or within 42 days of delivery remains largely unknown.
Lindley KJ, Madden T, Cahill AG, Ludbrook PA, Billadello JJ.
Obstet Gynecol. 2015 Aug;126(2):363-9. doi: 10.1097/AOG.0000000000000911.
Select item 26241425
Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: This is an excellent review of the patterns of behavior for prescribing and counseling regarding contraception in patients with congenital heart disease. This is specifically a review of prescribing behaviors in an ACHD center. This is a small study, only reviewing 100 women of childbearing age. 63 of whom reported using any contraceptive method whatsoever. 30 reported utilizing a method with a failure rate of <1% per year, which would include Depo-Provera, oral combination hormonal cyclic contraception, intra-uterine device, or hormonal patch or ring. 20 of the women documented using a method with much higher failure rates in the 6-12% per year range, which would likely be condoms. 9 of which utilized long-acting reversible contraception. 64 of 141 total pregnancies reported were unexpected. Only 1 pregnancy occurred with a tier I method of contraception. The high incidence of unintended pregnancy in this group may be related to underuse of highly effective methods of contraception. Specific counseling on tier I methods of contraception may be able to reduce unintended pregnancies in women with congenital heart disease. Tier III contraception includes condoms, withdrawal, rhythm method, sponge, spermicide, and fertility awareness, associated with failure rates of up to 18-28% per year. On average 85% of women are able to become pregnant within one year without contraception. The majority of women with congenital heart disease are sexually active, and an exceptional minority utilize Tier I methods of contraception. These US results are the first reported, that support the previously documented findings from prior European studies. It is imperative that all women with CHD receive counseling regarding the risks of pregnancy morbidity and mortality. As well all women need lesion/palliation appropriate counseling regarding the use of contraception, including the specific use of estrogen containing contraception if appropriate. Many patients with congenital heart disease have relative or absolute contraindications to pregnancy including pulmonary hypertension, heart failure, mechanical valve heart disease, and chronic use of teratogenic medical therapy. It is crucial that our patients, and their partners receive aggressive counseling and education regarding their risks of pregnancy additionally their risks of post-partum progression of disease and ability to care for or contribute to the care of children for their lifetime.
Take home points:
- Contraception counseling and pregnancy planning remains a very important aspect of adult congenital care.
- It is crucial to have this discussion as a team with the patient and partner.
- It is unfortunate that only an exceptional minority utilize Tier I methods of contraception.
Kuijpers JM, van der Bom T, van Riel AC, Meijboom FJ, van Dijk AP, Pieper PG, Vliegen HW, Waskowsky WM, Oomen T, Zomer AC, Wagenaar LJ, Heesen WF, Roos-Hesselink JW, Zwinderman AH, Mulder BJ, Bouma BJ.
Eur Heart J. 2015 Aug 14;36(31):2079-2086. Epub 2015 Apr 16.
Select item 26271472
Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: This is an excellent discussion presented by the Dutch research group interestingly adult men with a secundum type atrial septal defect have a worse survival expectancy than a gender-matched cohort from the general population. Male patients are also at higher risk of acquiring additional morbidities during adult life. The significance of gender disparity may warrant gender-specific guidelines for the long-term management of these patients. The CONCOR registry delivers again on another excellent dataset analysis. Survival of female patients with secundum-type atrial septal defect is comparable to other women in the population without an ASD. The dysrhythmias and conduction disturbances occurred beginning at a young age, and affect a large proportion of patients during their lifetime. The incidence of pulmonary hypertension, and congestive heart failure begin to increase at age 35 and increase progressively with age. They evaluated 1088 total patients, the ratio of men to women is 362 in men, and 726 women (1:2). Nearly 97% of the lesions were closed surgically prior to the year 2000. The average age at surgical repair was 10 years of age. The overall high morbidity of ASD2 (secundum ASD) patients necessitates appropriate surveillance and screening. The effect of gender on both mortality and morbidity was not confounded by closure status. Men are at higher risk for SVTs, conduction disturbances, cerebrovascular thromboembolism, HF and have reduced survival than women. Although the incidence of PAH is higher in women, mortality due to PAH is higher in men.
Take home points:
- Men are at higher risk for SVTs, conduction disturbances, cerebrovascular thromboembolism, HF and have reduced survival than women with ostium secundum ASD regardless of the closure status.
- Although the incidence of PAH is higher in women, mortality due to PAH is higher in men.
Dardi F, Manes A, Palazzini M, Bachetti C, Mazzanti G, Rinaldi A, Albini A, Gotti E, Monti E, Bacchi Reggiani ML, Galiè N.
Eur Respir J. 2015 Aug;46(2):414-21. doi: 10.1183/09031936.00209914. Epub 2015 May 28.
Select item 25810155
Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: Pulmonary arterial hypertension is a severe disease with a complex pathogenesis, for which combination therapy is an attractive option. This trial aimed to evaluate the impact of sequential combination therapy on both short-term responses and long-term outcomes in a real world setting. Patients with all cause PAH including CHD who were not meeting treatment goals on either first line bosentan or sildenafil monotherapy were given the addition of sildenafil or bosentan and then reassessed in 3-4 months. The overall survival estimates were 91% at 1 year and 59% at 5 years. There was documentation of significant short-term clinical and hemodynamic improvement in patients on dual therapy who fail monotherapy in a real world setting regardless of the etiology.
Take home points:
- There is significant short-term clinical and hemodynamic improvement in patients on dual therapy for PAH using Bosentan and Sildenafil who fail monotherapy in a real world setting regardless of the etiology.