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Case Study | CARDIAC IMAGING The Role of Cardiac MRI in Understanding Physiology of Pulmonary Hypertension in Complex CHD By Ramiah Rajesh Kannan, MD, DNB, PDCC, Associate Professor, and Mahesh Kappanayil, Associate Professor, Departments of Radiology and Pediatric Cardiology, Amrita Institute of Medical Sciences Signa* HDxt 1.5T Cardiac magnetic resonance imaging (CMR) is emerging as a powerful, non-invasive tool in assessment of congenital heart diseases (CHD). CMR comprises multiple tools that combine excellent anatomical visualization, with vital physiological information regarding blood flow and cardiac functions. It is especially valuable in assessing young adult/teenagers as well as adult patients with complex CHDs. Many such patients have pulmonary artery hypertension (PAH) as sequelae. Complexities of anatomy and/or physiology in an older patient often preclude adequate understanding through conventional imaging modalities like echocardiography and cardiac catheterization. CMR indices to accurately predict pulmonary artery pressures and resistances are yet to be adequately validated, especially in association with complex CHDs. However, combination of anatomical and physiological information obtained through CMR can aid understanding and decision making in complex situations. MR technique Cardiac MRI is done using a Signa HDxt 1.5T scanner from GE Healthcare with an 8-channel cardiac array coil. All 2D FIESTA (SSFP) sequences are done in suspended respiration in all 3 planes continuous sections across the heart in addition to routine short axis, long axis, and outlet views. 2D FIESTA sequence Gd-MRA sequence 2D phase contrast sequence Minimum TR Minimum TR and TE 20 degree flip angle 1.3 ms TE 25 degree flip angle 62.5 kHz bandwidth 60 degree flip angle 90.91 kHz bandwidth 4–6 views per segment 125 KHz bandwidth 256 X 256 matrix 128 X 128 matrix 160 x 160 matrix 2.6 mm slice thickness and ASSET factor of 2 4 NEX 12 views per segment and 8 mm slice thickness MRA is done with suspended respiration using fluoro triggering. GEHEALTHCARE.COM/MR 44 10 mm slice thickness and flow compensation Fast 2D phase contrast flow studies are done with free breathing. SPRING 2012 Case 1: Patient history Case 1 An 18-year-old patient, diagnosed with complex CHD in early Aorta childhood presented with effort intolerance, NYHA class II; SpO2 at 92%; single second-heart sound, continuous murmur Aorta over right scapular region. Patient had not undergone an operation for CHD. MR findings Cine 2D FIESTA sequence showed a large conoventricular Figure 1. septal defect with overriding aorta (Figure 1). Post contrast MAPCA to left Figure 2. MRA showed long segment pulmonary atresia with no native pulmonary arteries. There was a large (22 mm) aorto-pulmonary (AP) collateral (arising at T4 level), supplying branches to all segments of the left lung. MRA also showed a branch from the above collateral, stenotic close to its origin (6.5 mm), supplying the right lung with branches to all segments MAPCA to left of right lung (Figure 2). MRA also showed excellent arborization MAPCA to right of the right pulmonary vasculature derived from the AP collateral and rat-tailing and poor arborization of the left Figure 3. Figure 4. pulmonary arterial vasculature (Figure 3). Phase contrast sequences were used to study the blood flows across the aorta, the AP collateral to left and right Clinical interpretation lungs, the right and left pulmonary veins, and superior and inferior vena cavae. Patient is clinically well-preserved with SpO2 greater than Systemic flows (Qs) 4.9 liters/minute Total pulmonary blood flow (Qp) 9.6 liters/minute Right pulmonary blood flow 7.6 liters/minute Left pulmonary blood flow 2 liters/minute Qp:Qs Nearly 2:1 90%, with an overall Qp:Qs of nearly 2:1. Anatomically looked amenable to total correction with ventricular septal defect closure and right-ventricle-to pulmonary artery conduit. However flow studies (phase contrast) clearly showed that right lung accounts for 80% of the total pulmonary blood flow, despite being supplied by a stenotic collateral. The left lung, supplied by an unrestrictive, large collateral, shows low flows (20%) with flow-pattern suggestive of high Flow pattern in the left lung showed lower peak velocity vascular resistance. and rapid descent as compared to the right pulmonary Severe pulmonary vascular disease (Eisenmengerization) of flows (Figure 4). the left lung with preservation of right lung resistances. CMR flow studies clearly demonstrated unfavourable result for total surgical correction. GESIGNAPULSE.COM 45 SPRING 2012 Case Studies MAPCA to right Case Study | CARDIAC IMAGING Ramiah Rajesh Kannan, MD, DNB, PDCC is Associate Professor at Amrita Institute of Medical Sciences. Case 2: Patient history Phase contrast flow/time graphs of branch pulmonary artery flows show rapid-deceleration pattern of elevated pulmonary vascular resistance with left pulmonary blood A 16-year-old patient presented with mild effort intolerance, flow less than the right (Figure 7). an SpO2 of 85%, and an uncertain diagnosis on previous evaluations although truncus arteriosus was suspected. MR findings Cine 2D FIESTA showed a large conoventricular VSD, two balanced ventricles, and large pulmonary artery overriding the VSD (Figure 5). Other Cine images showed patent ductus arteriosus shunting right-to-left and sustaining systemic blood flow, and juxta ductal coarctation. 3D MR SVC flows 1.3 liters/minute IVC flows 2.1 liters/minute Systemic flow (Qs) = SVC + IVC 3.4 liters/minute LPA flows 2.7 liters/minute RPA flows 3.7 liters/minute Pulmonary flow (Qp) = RPA + LPA 6.4 liters/minute Qp:Qs 1.9 : 1 angiography revealed aortic atresia, hypoplastic ascending aorta, reduced arborization and peripheral pruning of Clinical interpretation bilateral lungs, more prominent in the left lung (Figure 6). Based on MRI findings, the patient was correctly diagnosed with hypoplastic ascending aorta with duct-dependent systemic blood flow. Phase contrast flow study revealed Qp:Qs of 1.9:1 with co-existing pulmonary vascular disease. CMR also indicated that left pulmonary vascular resistance is likely to be higher than the right. Case 2 MPA MPA LV Asc aorta RV Figure 5. GEHEALTHCARE.COM/MR Figure 6. Figure 7. 46 SPRING 2012 Case 3 Coarctation PDA Coarctation PDA MPA Figure 9. Case 3: Patient history Figure 10. Patient subsequently underwent bare-metal stenting of the coarctation, leaving PDA open. Post-stenting CMR showed mild increase in Qp:Qs to 1.2:1, with an increment of 34% in A 30-year-old patient presented with features of biventricular absolute pulmonary blood flow and moderate improvement failure and PAH, with upper body hypertension, differential in biventricular volumes and function. cyanosis, and differential clubbing. Discussion MR findings Cardiac MR is a useful tool in establishing morphological Cine 2D FIESTA demonstrated a large PDA shunting diagnosis in complex congenital heart diseases. A combination bi-directional with a tight pre-ductal coarctation of of CMR tools such as Cine (SSFP) images, MR angiography, Aorta and severe biventricular systolic dysfunction. and phase contrast (flow) studies allow unique insight into 3D MR angiography showed the complex relationship patterns and physiology of CHD-PAH. CMR also overcomes between the PDA and coarctation (Figures 8, 9). the shortcomings of cardiac catheterization in accurately Phase contrast flow/time graphs showed a pattern of PAH assessing shunts and differential pulmonary blood flow in in both lungs, with lower flows in the right lung and Qp:Qs complex cardiac lesions with PAH. of 0.9:1 (Figure 10). PDA showed a systolic right-to-left shunt and a pan-diastolic left-to-right shunt (net flow being about 0.3L/min right-to-left). Mahesh Kappanayil, FNB, Pediatric Cardiology In this case, CMR clearly showed the complex anatomy of PDA and aortic coarctation. In addition, the Cine images and is an Associate Professor at Amrita Institute of Medical Sciences. phase contrast flow study provided the detailed physiology of circulation in the pulmonary arteries and aorta, which was used to guide further treatment plans. Ramiah Rajesh Kannan, MD, DNB, PDCC, is Associate Professor at Amrita Institute of Medical Sciences. He has more than five years of experience in cardiac imaging and has performed more than 5,000 cardiac CT angiography scans (adult coronary and CHD) and more than 500 cardiac MRI exams. Mahesh Kappanayil, FNB, is an Associate Professor at Amrita Institute of Medical Sciences. He completed his medical graduation (MBBS) from University of Delhi in 1995, and subsequently his residency in Pediatrics. He completed his post-doctoral fellowship in Pediatric Cardiology from Amrita Institute of Medical Sciences (AIMS), Kochi, Kerala, which is one of the leading pediatric cardiac care centers in Asia. He has continued as a faculty member at AIMS since completion of his fellowship in 2006. Amrita Institute of Medical Sciences in Cochin Area, India brings together a dedicated team of physicians, nurses, and other healthcare professionals to provide the highest standards of medical treatment. The full range of primary and specialty care medical services enables cross-specialty consultation, which assures outstanding treatment for each patient. The extensive infrastructure offers extensive facilities comprising 25 modern operating theatres, 210 equipped intensive-care beds, a fully computerized and networked Hospital Information System, a fully digital radiology department, a 24/7 telemedicine service, and a NABL accredited clinical laboratory. GESIGNAPULSE.COM 47 SPRING 2012 Case Studies Figure 8.