Abstract 4686: Noncontrast Three Dimensional Dark Blood Magnetic Resonance Imaging in Adult Congenital Heart Disease Patients After Transcatheter Intervention

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Marshall W Winner ◽  
Subha V Raman ◽  
Orlando P Simonetti ◽  
Yiu-Cho Chung ◽  
Lindsay Arnott ◽  
...  

Background : Adults with congenital heart disease (ACHD) often require transcatheter therapies as a result of residual lesions. CT is well-suited for evaluation of stents but exposes young adults to radiation. Gradient echo MRI suffers from artifact limiting assessment of stented segments. We investigate the use of a novel 3D turbo spin echo (TSE) MRI sequence in these patients. Methods: ACHD patients presenting for MRI with prior interventions underwent an additional T1-weighted 3D dark blood navigator respiratory gated TSE scan on a1.5 Tesla system. Type of CHD, prior CT, radiation dose, length of time between examinations, and luminal diameters on each study were obtained. Continuous variables analyzed using Student’s t-test and Bland-Altman plots. Results: Eleven patients underwent both MRI and CT. Diagnoses were coarctation of the aorta (n=6) and tetralogy of Fallot (n=5) with intervention in the aorta or pulmonary arteries respectively. Average radiation dose was 19.57mSv; average time between CT and MRI was 99 ± 160 days. Luminal diameters of stented vessels correlated closely between TSE MRI and CT (r 2 = .85) with a small bias toward overestimation with MRI (mean 22.4 +/− 4.3 mm and 20.9 +/− 3.7 mm, p< .01). There were no cases of in-stent stenosis demonstrated by CT. Conclusion: Metallic artifact is significantly reduced with 3D navigator respiratory gated TSE MRI allowing visualization of anatomy in the vicinity of stents, providing a potential method for assessment of complex anatomy without radiation exposure or use of contrast agents. Further studies of this technique are warranted, particularly in patients with in-stent stenosis.

Heart ◽  
2017 ◽  
Vol 104 (9) ◽  
pp. 732-737 ◽  
Author(s):  
Clare Arnott ◽  
Geoff Strange ◽  
Andrew Bullock ◽  
Adrienne C Kirby ◽  
Clare O’Donnell ◽  
...  

ObjectiveEisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT).MethodsFrom January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand. Demographic, medical and outcome data were collected and analysed prospectively and retrospectively.ResultsThe patients with ES were predominantly female (60%), aged 31 (SD 12) years. At diagnosis of ES, 64% were WHO functional class ≥3. The most common underlying lesion was ventricular septal defect (33%) with 21% having ‘complex’ anatomy. Over a median follow-up time of 9.1 years, the majority (72%) had been prescribed at least one AT (49% single agent), mostly bosentan (66%, 168 patients). The mean time on AT was 6 (SD 3.6) years. Those on AT were more functionally impaired at presentation (69% WHO ≥3 vs 51%, p=0.007) and more likely to have been prescribed anticoagulation (47% vs 27%, p=0.003). The risk of death/transplant was 4.8 %/year in AT exposed versus 8.4% in those never exposed. On multivariable analysis, exposure to AT was independently associated with greater survival (survival HR 2.27, 95% CI 1.49 to 3.45; p<0.001). WHO ≥3 at presentation was associated with a worse prognosis (mortality HR 1.82, 95% CI 1.19 to 2.78; p=0.006).ConclusionTreatment with AT was independently associated with greater survival in patients with ES, even though they were comparatively sicker prior to treatment.


Author(s):  
Philip Kilner ◽  
Ed Nicol ◽  
Michael Rubens

Both CMR and MSCT give almost unrestricted access to intra-thoracic structures, whereas ultrasonic access may be limited in ACHD patients. MSCT, generally using intravascular contrast, gives superior spatial resolution more rapidly than CMR, although the radiation dose is a concern in younger patients who may require repeated studies. MSCT gives better visualisation of epicardial coronary arteries and small collateral vessels, and can show conduit calcification or stent location clearly. It provides an alternative to CMR in patients with a pacemaker or ICD. CMR offers unrivalled versatility of acquisition methods without ionizing radiation, enabling measurements of biventricular function, flow, myocardial viability, angiography and more. A dedicated CMR service should be available in a centre specializing in ACHD care. Appropriate understanding is needed for the evaluation of congenitally and surgically altered circulatory function, for example after Fontan operations, surgery for transposition of the great arteries or tetralogy of Fallot.


2017 ◽  
Vol 11 (4) ◽  
pp. NP27-NP30
Author(s):  
Billie-Jean Martin ◽  
Ivan M. Rebeyka ◽  
Jeffrey F. Smallhorn ◽  
Dylan A. Taylor ◽  
Isabelle F. Vonder Muhll

Adult patients with repaired congenital heart disease are presenting with previously unseen types of residual lesions and consequences of prior repair. Patients with d-transposition of the great arteries repaired with atrial switch operations are returning with dysrhythmias and atrioventricular valve disease requiring intervention. We present the challenging case of a young adult with a residual shunt identified on preoperative three-dimensional transthoracic echocardiography, the precise anatomy of which was only characterized intraoperatively.


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