Lu CW, Shih JC, Chen SY, Chiu HH, Wang JK, Chen CA, Chiu SN, Lin MT, Lee CN, Wu MH.
Circ J. 2015 Jun 25;79(7):1609-17. doi: 10.1253/circj.CJ-14-1368. Epub 2015 May 9.
PMID: 25959432 Free Article
Select item 26115543
Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: This article addresses an issue that all practitioners who care for our pregnant patients struggle with. We have three excellent, well-studied risk calculators available for our use. With each pre-natal consult we carefully calculate the risk, and then attempt to translate the results of these clinical calculators into practical advice that our patients can relate to. Although this retrospective study looks primarily at women in Taiwan in a relatively homogenous population their results are encouraging. The authors studied 268 pregnancies in 190 women with CHD and18 (6.7%) had cardiac complications. It would appear that the CARPREG risk score, the ZAHARA risk score, and the modified WHO classification are all relevant to our practice and there is not wide variability in the applicability or ability to risk stratify our patients. CARPREG is a Canadian risk calculator. ZAHARA is a European collaborative study. The modified WHO is recommended by the ESC. CARPREG utilizes 4 risk predictors: prior cardiac event, NYHA functional class >II or cyanosis at baseline, impaired systemic ventricular function, or severe left sided obstruction. ZAHARA was designed specifically for women with congenital heart disease, and includes 8 risk predictors: history of arrhythmia, cardiac medications prior to pregnancy, NYHA class >II, severe left heart obstruction, severe systemic AV valve regurgitation, severe pulmonary AV valve regurgitation, mechanical valve prosthesis and cyanotic heart disease. CARPREG and WHO can be applied to all women. The study was divided into cardiac events, obstetric events, and fetal events. Their final conclusion is that the modified WHO may be able to better predict cardiac outcomes of pregnancy in patients with CHD, but all three risk scores were effective. An App that would permit calculating all three scores with just a few data points would be very useful.
Take home points:
- ZAHARA score was designed specifically for women with congenital heart disease.
- The modified WHO classification is the only method that takes into account both specific heart lesions (eg, Marfan syndrome, bicuspid aortic valve, tetralogy of Fallot, aortic coarctation, Fontan circulation or systemic right ventricle) and the clinical cardiac status (eg, systemic ventricular dysfunction).
- Modified WHO may be able to better predict cardiac outcomes of pregnancy in patients with CHD, but all three risk scores were effective.
Itagaki S, Chikwe JP, Chiang YP, Egorova NN, Adams DH.
J Am Coll Cardiol. 2015 Jun 9;65(22):2363-9. doi: 10.1016/j.jacc.2015.03.575.
Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: For too long we have been tending to equate bicuspid aortic valve disease and associated aortopathy with Marfan syndrome aortopathy. This paper nicely outlines the risks of continuing to treat all aortopathies with the same algorithm. This paper argues for the development, appropriately, of distinct algorithms for the varying kinds of aortopathies including Marfan syndrome, Vascular Ehlers Danlos, SMAD, and ACTA, as well as the infinitely more common, and less likely to progress bicuspid aortic valve. Dr. Adams and his colleagues at Mt. Sinai have brought to light a gap in care between internal medicine trained cardiologists, and congenitally trained cardiologists. The notable findings include that the long-term incidence of thoracic aortic dissection is 10-fold higher in patients with Marfan syndrome compared to those with bicuspid aortic valve, while patients with bicuspid aortic valve are only at 20% greater risk of dissection than an age-matched normally structured aortic valve control group. We should not extrapolate operative management of patients with bicuspid aortic valve from Marfan data. One of the limitations of this study was that patients who underwent planned thoracic aortic procedures were excluded, and that therefore limited the inclusion of many of the initially identified Marfan patients. During the 2-year follow up period of the study half of the BAV patients were non-progressors while the majority of the Marfan patients were progressors.
Take home points:
- The risk of dissection is 10-fold higher in patients with Marfan syndrome compared to those with bicuspid aortic valve, while patients with bicuspid aortic valve are only at 20% greater risk of dissection than an age-matched normally structured aortic valve control group.
- A common algorithm should thus be avoided while managing a spectrum of aortopathies of varying etiologies.
- Morbidity even after aortic valve replacement in Marfan syndrome is substantially high with long-term risks for thoracic aortic dissection, new thoracic aortic aneurysms, and need for thoracic aortic surgery compared with patients with bicuspid aortic valves.
Lewis G, Thorne S, Clift P, Holloway B.
Clin Radiol. 2015 Jun;70(6):667-75. doi: 10.1016/j.crad.2015.02.011. Epub 2015 Mar 26. Review.
Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: This single-center commentary is particularly valuable in reviewing cardiac MRI (CMR), and cardiac CT protocols in Fontan patients. For CMR the protocol should consist of planned anatomical sequences including the whole chest with black blood spin-echo or steady state free precession (SSFP) sequences in the 3 orthogonal planes and cine balanced SSFP sequences in the long and short axis through the single ventricle. This allows assessment of ventricular function. The cine b-SSFP and phase contrast sequences through the ventricular outflow tract, Fontan circuit, and branch PA’s are essential in evaluating obstruction, stenosis, or energy sinks. Time-resolved MRA may be valuable to assess flow dynamics in experienced operators hands. CT is useful for evaluating acute or chronic complications including: Pulmonary embolism, AV malformations, and venous collaterals. Due to Fontan hemodynamics contrast delivery should be considered pre-procedure. Opacified blood within the pulmonary arteries mixes with non-opacified blood from the IVC more quickly than with typical anatomy. This leads to heterogenous enhancement with standardized PE protocols that is easily misinterpreted at a filling defect, or pulmonary embolus. A dual arm contrast injection, with a delayed phase image acquiring may allow for homogenous contrast uptake, but there is a tradeoff of decreased contrast concentration. Additional, consideration in patients with an interrupted IVC, and Kawashima would not benefit from the same strategy.
Take home points:
- Regular follow-up with MRI, and CT as an adjunct in patients with Fontan circulation is now increasingly utilized.
- Important radiologically identifiable complications include thromboembolism, pulmonary artery stenosis and pulmonary vein compression, hepatic venous congestion, and cirrhosis and shunting.