Cardiovascular Imaging Interactive Case Discussions

Transcription

1 Cardiovascular Imaging Interactive Case Discussions Satinder Singh MD, FCCP Professor of Radiology & Medicine (Division of CV Diseases) Chief Cardiopulmonary Radiology Director Cardiac CT UAB SCBT.MR September 17-21, 2017

2 Case 1: 17 Y/M with murmur on routine well child visit lead to abnormal echo

3 What is the diagnosis? 1. Coronary artery fistula 2. Occluded RCA with collaterals 3. Anomalous coronary artery origin 4. Sinus of valsalva aneurysm

4 Anomalous origin of RCA from pulmonary artery (ARCAPA) A rare congenital coronary artery anomaly It may be associated with other congenital heart defects Patients with ARCAPA may remain asymptomatic until adulthood, continuous murmur in asymptomatic patient common presentation May cause exertional chest pain, syncope, sudden cardiac death, or ischemic cardiomyopathy Surgical correction MPA Ao MPA Ao

5 ALCAPA Bland-White-Garland syndrome 1 in 300,000 live births Usually symptomatic in infancy/early childhood (6-8 wks) Coronary steal phenomenon; signs of left ventricular ischemia and congestive heart failure Surgical repair: Reimplantation Tunnel operation (Takeuchi Repair) Ao PA

6 Coronary Artery Anomalies Anomalies of origin High takeoff Multiple ostia Single coronary artery Anomalous origin from pulmonary artery Origin from opposite or non coronary cusps Anomalies of course Interarterial Prepulmonic Intraseptal Retroaortic Anomalies of termination Coronary artery fistula Coronary arcade Extracardiac termination PA Ao

7 6.3 million 18 year old military recruits, intense military training x 8 wks Sudden non traumatic death rate 13.0 per recruit-years 51% cardiac causes (61% coronary artery abnormalities, 20% myocarditis, 13% hypertrophic cardiomyopathy) An anomalous left coronary artery accounted for 1/3 of the cases Hemodynamic changes during maximum exertion sports activities: HR: increases from 65 to 185 CO: fro 5 to 22 L/mt SV: from 77 to 122 cc Eckart E.R. et Al, Ann Intern Med. 2004;141:

8 47 y/o with syncope & exercise-induced transient ventricular tachycardia, inability to cannulate RCA MPA Ao R L NC Normal

9 Inter arterial course High risk for sudden cardiac death due to Slit like opening Acute angle of ostium Stretch of intramural segment Compression between commissure of right and left coronary cusp

10 Surgical: Treatment of Inter-arterial course Unroofing: Promising for intramural course May cause aortic insufficiency Re-implantation into appropriate sinus: Risk of subsequent ostial stenosis Long term data lacking CABG: Problem of competitive flow Ligation of native vessel

11 Case 2: 63 Y/F with hyperlipidemia and intermittent sub sternal chest pain x 3 months. Exercise stress test reported normal. Echo bubble study was positive

12 What type of ASD is present? 1. Septum secundum 2. Sinus venosus 3. Septum primum 4. Coronary sinus unroofing

13 Sinus Venosus ASD (inferior) RA RV PV IVC LA LV RPA IVC LA RA LV

14 Atrial Septal Defect Secundum (60%) Level of fossa ovalis Excessive reabsorption of septum primum Adults Primum (30%) Lower atrial septum Deficiency of endocardial cushion tissue Associated with AV canal defect Often in neonates Sinus Venosus (10%) High at the level of SVC or low at IVC level Error in incorporation of the sinus venosus chamber into the RA Associated with PAPVR Adults Coronary sinus unroofing RA yale.edu LA CS CS unroofing

15 Sinus Venosus ASD Result from abnormal resorption of the embroyonic sinus venosus Superior (common): most common in upper atrial septum, rostral and posterior to FOV RUL PAPVR Inferior (rare): junction of IVC and right atrium RLL PAPVR svc LA Superior SV RA IVC LA PV Inferior SV

16 Inferior Sinus venosus ASD Very rare & difficult to diagnose Unexplained volume overload of right sided chambers should prompt for careful evaluation for: sinus venosus type of ASDs (superior or inferior) coronary sinus type of ASD (absence of roof of coronary sinus) partial anomalous pulmonary venous drainage Can result in right to left shunt with some degree of systemic oxygen desaturation Need surgical repair Circulation. 2013;127:

17 Case 3: 62 Y/F with unknown heart defect (hole in heart), increasing SOB and hypoxia. CT done for PTE

18 Regarding the heart anomaly, which of the following is accurate 1. Commonly present in adults 2. Acyanotic disease 3. Right aortic arch is always present 4. Risk of bacterial endocarditis

19 Unrepaired TOF 10% of untreated patients live more than 20 years Underdevelopment of pulmonary infundibulum due to unequal positioning of conus truncus causing valvular or subvalvular RVOT stenosis, subaortic VSD, overriding aorta & RV hypertrophy Most common cyanotic heart disease Hyper cyanotic spells with dyspnea on exertion relieved by squatting position, clubbing, CHF (with large VSD), endocarditis Boot shaped heart, decreased pulmonary vascularity Surgically repaired Unrepaired TOF with infundibular, pulmonary valve and main PA stenosis, Rt arch & mirror branches

20 TOF Treatment Palliative: (to increase pulmonary blood flow) Blalock-Taussig shunt: Subclavian artery to PA Modified BT shunt: Interposition Goretex graft between SCA and PA Waterson shunt: Ascending aorta to main or RPA Potts: Descending aorta to LPA Complete repair: (usually in first year of life) VSD closure Relieve RVOT obstruction: valvotomy, resection of infundibular muscle, RVOT patch, transannular patch, extracardiac conduit between RV and PA

21 Evaluation of Repaired TOF In treated TOF role of CT is to evaluate: Patency of shunt Status of pulmonary artery and branches Detect RV enlargement due to chronic volume overload Associated coronary artery anomalies Case Study: 33 Y/M, repaired TOF with hemoptysis Right arch with mirror branching Left subclavian ligated (prior BT) Narrowing of central PA Large MAPCA opacified RV hypertrophied PAHTN RUL cavity RV

22 Case 4: 57 Y/M with known CHD, chest pain

23 What is the diagnosis? 1. Tricuspid Atresia 2. D-TGA 3. L-TGA 4. TOF

24 Congenitally corrected TGA (L-TGA) Atrio-ventricular and ventriculo-arterial discordance Associated conditions: VSD (40-60%) Pulmonary stenosis (25%) Dextrocardia (20%) Coarctation (5%) Systemic RV will gradually fail PA AO Normal Ventricular function evaluation required as follow up LV LA RV PA Ao

25 Transposition of Great Vessels (D-TGA) Most common cyanotic CHD presenting in neonates Concordant atrioventricular & discordant ventriculoarterial connections Aorta is anterior connected to RV PA is posterior and connected to LV Must have mixing defect (intra or extracardiac) to survive

26 Relationship of Aorta & PA AO PA AO D-TGA AO PA Primitive Truncus A PA AO PA L-TGA R L PA AO PA PA P PA AO Normal AO AO Situs Inversus

27 Case 5: 34 Y/F with increasing SOB

28 D-TGA S/P Atrial Switch Atrial baffle redirects systemic blood to anatomic pulmonary LV and pulmonary venous blood to anatomic systemic RV Mustard: pericardial or tissue graft used for baffel Senning: Atrial wall & septum is reconstructed to form baffel Complications: Baffel stenosis/leak, arrythymia, RV failure, sudden death Ao MPA LA RV LV F

29 Arterial Switch (Jatenne) More preferred method now: Aorta and PA transected above valves and moved to correct circulatory position CA excised from rt side with button like margin and implanted on the lt side of heart Echo evaluation limited due to retrosternal position of the aorta and PA Dilatation of the great arteries and PA is common (PA hugging Ao) Information needed on F/U (complications): Any PA narrowing in between sternum and aorta Coronary artery evaluation after reattachment Evaluation of ventricle function RPA Ao LPA

30 Why radiologist need to know about CHD? May be discovered incidentally: ED visit due to chest pain, arrythymia or SOB CT may be done to evaluate post operative anatomy or suspected complications 45 Y/M, HTN, ED visit for Chest Pain, evaluate dissection NC R L AO LV LA No dissection Cor triatrium Anomalous Cx arising from right cusp with retro aortic course

31 Imaging Modalities Echo Excellent in infants Limited in adults: Poor acoustic window Chest wall abnormality Pectus deformity Kypho scoliosis Multiple thoracotomy scars Suboptimal depiction of extra cardiac vasculature Cath Hemodynamics Only when intervention needed CMRI Excellent anatomic & functional information Limited: Pts with permanent pacemakers, AICD & occluder devices Not widely available MDCT: Quick, widely available, high spatial resolution, isotropic imaging, hardware not problem, extracardiac abnormalities Limitations: No physiological information Radiation Contrast

32 Conclusion Thorough understanding of normal anatomy, morphologic features of CHD, knowledge of surgical procedures and their complications Try to get clinical information especially surgical details CT protocol may need to be modified: Gating if looking for coronary arteries or small structures Don t use saline chaser May need to inject upper & lower extremity or get delayed phase imaging Use sequential approach for CTA analysis >85% of infants with CHD now reach adulthood Need lifelong care & imaging evaluation; reoperations from underlying anatomy or previous surgical palliative procedures YOU WILL SEE THESE DISEASES

33 THANK YOU