S. Bruce Greenberg, MD FNASCI and President, NASCI Professor of Radiology and Pediatrics University of Arkansas for Medical Sciences

Transcription

1 S. Bruce Greenberg, MD FNASCI and President, NASCI Professor of Radiology and Pediatrics University of Arkansas for Medical Sciences

2 No financial disclosures

3 Aorta Congenital aortic stenosis/insufficiency Aorta coarctation Right ventricle size Anomalies of the pulmonary artery or valve Tetralogy of Fallot or Truncus arteriosus Complex palliations Univentricular heart Hypoplastic left heart syndrome

4 Congenital aortic stenosis/insufficiency Collagen vascular disease 41 year old woman 18 year old man with Marfan disease with bicuspid aortic valve

5 Prophylactic aorta root replacement 5.0 cm Aorta diameter < 5.0 cm Rapid growth > 1.0 cm/year Family history premature aortic dissection Moderate or greater aortic regurgitation MRI or CT Annual if abnormal beyond aorta root 5 years if normal beyond aorta root Milewicz DM, et al. Treatment of aortic disease in patients with Marfan Syndrome. Circulation 2005; 111: e150-e157. Radke RM, et al. Diagnosis and treatment of Marfan syndrome: an update. Heart 2014; 100:

6 Diameter up to 2 mm/year Annual follow up recommended: Aorta dilatation > 4.5 cm Rapid change in aorta diameter + family history of aortic dissection Longer interval in others

7 Valve surgery 61% replace aorta if diameter 4.5 cm No valve surgery 55% replace aorta if diameter 5.0 cm 20 year old with bicuspid aortic valve & ascending aorta aneurysm Verma S, et al. Knowledge, attitudes and practice in surgical management of bicuspid aortobpathy: a survey of 1000 cardiac surgeons. J Thorac Cardiovasc Surg 2013; 146:

8 42 year old with history of coarctation repair

9 Associations Bicuspid aortic valve Patient age No BAV and age < 20 years 3% BAV and age > 39 years 50% Oliver JM, et al. Risk factors for aortic complications in adults with coarctation of the aorta. J Am Coll Cardiol 2004; 44:

10 50% re-coarctation cases: no clinical findings MRI re-coarctation detection rate same for symptomatic & asymptomatic patients Aorta aneurysms are not detected by echo

11 Lifelong cardiology follow-up is recommended for all patients with aortic coarctation (repaired or not), including an evaluation by or consultation with a cardiologist with expertise in ACHD. Evaluation of the coarctation repair site by MRI/CT should be performed at intervals of 5 years or less, depending on the specific anatomic findings before and after repair. Warnes CA, et al. ACC/AHA 2008 guidelines for management of adults with congenital heart disease Circulation 2008: 118: e714-e833

12 Anomalies of the pulmonary artery and valve Tetralogy of Fallot and Truncus arteriosus Transposition complex

13 Pulmonary regurgitant fraction 25% Right ventricle enlargement Abnormal cardiac function QRS duration > 140 msec Associated anatomic abnormalities Geva T. Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. Journal of Cardiovascular Magnetic Resonance 2011; 13: 9

14 Systolic function RV ejection fraction < 47% LV ejection fraction < 55% Diastolic dysfunction Abnormal tricuspid flow patterns Late diastolic forward flow Geva T. Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. Journal of Cardiovascular Magnetic Resonance 2011; 13: 9

15 RV EDVI > 150 ml/m 2 (z-score > 4) Right ventricle remodeling threshold limit > 170 ml/m 2 (Therrien et al. Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot. Am J Cardiol (2005) 95: ) > 160 ml/m 2 (Oosterhof et al. Preoperative thresholds for pulmonary valve repalcement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance (2007) 116: ) > 150 ml/m 2 (Dave et al. Early insertion of a pulmonary valve for chronic regurgitation helps restoration of ventricular dimensions. Ann Thorac Surg (2005) 80: )

16 Univentricular heart Hypoplastic left heart syndrome

17 Kaplan-Meier plot of cumulative freedom from the end point (death or transplantation stratified by indexed end-diastolic volume (EDV) > 125 ml/bsa 1.3 (P<0.001) Right ventricle ejection fraction 38% Right ventricle end diastolic volume 265 ml RV end diastolic volume index 156 ml/m 2 Rathod RH, et al. Cardiac magnetic resonance parameters predict transplantation-free survival in patients with Fontan circulation. Circ Cardiovasc Imagning (2014) 7:

18 Aorta aneurysm: MRI and CT diameter measurements Right ventricle regurgitation: RV EDVI for valve replacement Univentricular heart palliation: RV EDVI for survival prognosis