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A tale of 3 patients... A short circuit, a steal and the blues Rami Khouzam, MD May 20, 2005.

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Presentation on theme: "A tale of 3 patients... A short circuit, a steal and the blues Rami Khouzam, MD May 20, 2005."— Presentation transcript:

1 A tale of 3 patients... A short circuit, a steal and the blues Rami Khouzam, MD May 20, 2005

2 Case 1 –A 65 yo AAM with a PMHx. significant for a recent NSTEMI, HTN and ESRD, presented with chest tightness. –Troponin I: 53 NG/ml (N < 1.5 NG/ml). –Cardiac catheterization: Lt Cx bifurcation lesion at the level of OM 2, and multiple RCA lesions not amenable to intervention.

3 EKG 1

4 EKG 2

5 What Now? A) Take patient again to the cath. lab B) Start GIIbIIIa inhibitors C) Repeat testing of cardiac enzymes D) Continue same management E) None of the above

6 Misinterpretation of ECG due to Cable Malfunction: A Newly Described ECG error. Fluid contamination at the trunk cable connector  impedance change on the V leads’ cable  leading monitor to view V2 through V6 as the same point electrically  the cable was subsequently changed J Electrocardiol, 2005;38:210-211

7 Case 2 Where is the Justice?

8 Case Report O.C. is a 70 y.o. woman who presented with syncope (several episodes in past) Denied CP, palpitations, prodrome, incontinence, visual changes, aura, tongue- biting, or post-ictal state PMH: HTN, CVA (6 yrs ago), GERD PSH: CABG 1996, Hysterectomy SH: occ TOB FH: CAD, DM Meds: Accupril, ASA, Prevacid

9 PE: T 98.9 o P 66 R 16 BP 190/64 HEENT: unremarkable. Neck: supple without JVD or bruits. Chest: CTAB. CVS: RR @ ~60, nl S1/S2, no S3, +S4; PMI NDP II/VI SM @ RUSB -> neck; I/IV DM @ 3 rd L ICS. Abdomen and Neurological exams: unremarkable. Ext: No edema. Peripheral pulses 2+ throughout except R radial and brachial pulses Orthostats (L arm): supine 210/90, 52; standing 220/100, 56 R arm BP 110/80 mmHg

10 /unequal_UL_pulses.htm Differential Dx of Unequal Pulses Thoracic Outlet Syndrome Arteritis (Takayasu’s or temporal) Embolism Raynaud’s syndrome Thoracic Aortic Aneurysm Thromboangiitis Obliterans Aortic Dissection Coarctation of the Aorta Syphilitic Aortitis Abnormal Vessel Development Iatrogenic (e.g., trauma following catheter studies) Blalock Shunt

11 CMP/CBC/CIEs/EKG/CXR/CT head: all WNL Echo: moderate aortic valve disease (AV area ~1.35 cm 2, gradient ~50 mmHg) AR, AS with intact LV function (EF 65%) Carotid duplex USG: R subclavian steal and 60% stenosis of R ICA, 30% stenosis of R ECA Arteriogram: tortuous lesion of the proximal R subclavian with an anomalous retroesophageal course Axillary-carotid bypass recommended

12 McIntyre, K., Subclavian Steal Syndrome, eMedicine, Oct 2003. Subclavian Steal Syndrome SSS is caused by occlusion of the proximal subclavian artery with subsequent retrograde filling of the subclavian artery via the vertebral artery Definition of SSS must include 1) neuro symptoms due to cerebral ischemia initiated by ipsilateral arm exercise, and 2) diminished BP in the ipsilateral arm due to stenosis or occlusion of the subclavian artery proximal to the vertebral artery origin



15 Arch aortogram

16 MRA

17 McIntyre, K., Subclavian Steal Syndrome, eMedicine, Oct 2003 Atherosclerosis is the most common cause of proximal subclavian artery lesions Risk factors include age, sex, FH, smoking, DM, HTN, HPL, hyperhomocystinemia Although retrograde blood flow in the vertebral artery is usually noted angiographically with proximal ipsilateral subclavian artery occlusion, subclavian steal may also occur with hemodynamically significant subclavian artery stenosis

18 El-Mallakh, R.S., Subclavian Steal Syndrome, Ferri's Clinical Advisor, 2004. On the right side, only a small distance separates the bifurcation on the subclavian artery and the origin of the vertebral artery; hence, the condition occurs less commonly on the right

19 McIntyre, K., Subclavian Steal Syndrome, eMedicine, Oct 2003. Pathophysiology Symptoms via flow-related phenomena Collateral vessels from the subclavian artery enlarge when the lesion in the proximal subclavian artery progresses The upper extremity becomes dependent on collateral vessels distal to the obstruction Collaterals serve as points of entry for retrograde flow to the arm from the head, shoulder, and neck

20 McIntyre, K., Subclavian Steal Syndrome, eMedicine, Oct 2003. When arm is exercised, vessels dilate to enhance perfusion to the ischemic muscle and resistance is lowered in the outflow vessels Blood is siphoned from the head/neck/shoulders through collaterals to supply this low-resistance vascular bed satisfying increased O 2 demand by exercising muscles of the arm Resistance in the outflow vessels of the arm increases when exercise ceases Retrograde flow in the vertebral artery reduced

21 Chan-Tack, K., Subclavian Steal Syndrome: A Rare but Important Cause of Syncope, SMA, Apr 2001. In Essence… Development of (-) pressure gradient between vertebrobasilar and vertebral- subclavian artery junctions Subsequent retrograde filling of the subclavian artery via the vertebral artery causes the subclavian artery to “steal” blood from the vertebrobasilar system

22 Clinical: Signs & Symptoms  Muscle cramping  Cerebral ischemia - Dizziness - Syncope - Dysarthria - Visual loss - Diplopia - TIA’s  BP differences


24 El-Mallakh, R.S, Subclavian Steal Syndrome, Ferri's Clinical Advisor: Instant Dx and Treatment, 2004. Proximal subclavian occlusion or stenosis CANNOT be present in the absence of a significant difference in BP between patient’s arms Therefore, A SIMPLE PHYSICAL EXAM can effectively eliminate significant subclavian arterial lesions without using angiography or duplex ultrasonography

25 Case 3 Out of the Blues 18 yr AA woman brought to ER: found comatose at home. Slurred speech, MS changes & inability to move left arm 6 wks prior to admission she had delivered a healthy 32 week old infant vaginally

26 Delivery complicated by profuse blood loss 2ry. to ruptured vaginal condylomas   hematocrit from 49% to 32% Exercise intolerance, severe fatigue, SOB: improved after a blood transfusion

27 Past Medical History Prolonged hospitalization at birth for almost one year, but no hx of cardiac surgery Some developmental delay  followed by a relatively active life & No limitation in daily activities Clubbing of fingernails and occasional cyanosis noted by family

28 Pertinent PE: Vitals: P:81, BP:108/54, RR:10 CVS: Single S2, Normal S1, RR @ 80 ø m, g, r No JVD Lungs: CTA bilat. ø w, c, r Ext: clubbing 3rd degree (fingernails & toenails) +/- cyanosis ø edema Neuro: Lt. flaccid hemiparesis: Lt. Arm > Lt. leg

29 Current Admission In ER: O 2 sat. : 65 % ABGs: 7.43/ 29/ 41/ 21/ 74% (post-intubation on 100% O 2 )

30 Differential Diagnosis Right-to-left shunt: - Anatomic shunts of systemic venous blood into the arterial circulation - Congenital heart diseases - Pulmonary arteriovenous fistula - Multiple small intrapulmonary shunts

31 EKG

32 Chest X-ray: Heart size normal No lung infiltrates or interstitial edema CT of the head without contrast: Normal

33 TTE (+ contrast echo): Agitated saline from right femoral vein


35 TTE (+ contrast echo): Agitated saline from upper extremity vein


37 Cardiac cath. Lab: PA catheter from right femoral vein (to left atrium & left ventricle) PA catheter from right subclavian vein (to right atrium, right ventricle & pulmonary artery)

38 CT-angiogram + coronal reformation, volume rendered color images: Injection of dye via right femoral vein


40 MRI/MRA (few days later): Hypoplastic vertebro-basilar system Area of hypersensitivity in pons  suggesting infarct

41 Le Bonheur Hospital: Surgery (Atrial septostomy with repair and transfer of the IVC drainage from left atrium to right atrium)


43 Outcome O 2 sat. 95 - 100 % on RA Stable 10 months after D/C No effort intolerance, No DOE

44 In the embryo, the sinus venosus receives the cardinal, umbilical, and vitelline veins. It communicates with primitive atrium via an orifice that has a right and left valve. Normally, the sinus venosus migrates to the right, and the left valve disappears. Anomalous Drainage of IVC to the Left Atrium

45 The right valve usually decreases in size, becoming the crista terminalis, Eustachian and Thebesian valves. If the right sinus venosus valve persists and fuses with the superior part of the septum secundum, the result is: An Inferior Vena Cava draining into the Left Atrium

46 Pathophysiology If one of the cavae enters the left atrium, the right side of the heart is effectively bypassed so that the right ventricle output and pulmonary blood flow should fall Meadows, W.R. Am. J. Cardiol. 1965 The left ventricular output theoretically remains unchanged because the increment in anomalous caval flow into the left atrium is matched by a reciprocal decrement in pulmonary venous flow into the left atrium

47 IVC normally carries about twice the volume of blood as the SVC Accordingly an anomalously draining IVC delivers a much larger proportion of systemic venous return to the left atrium than an anomalously draining SVC Perloff. 1994

48 The History Cyanosis from birth or infancy Survival into adulthood is the rule with recorded cases in the 6th, 7th & 8th decades Absence or paucity of symptoms in patients with cyanosis Davis, W.H. Br. Heart J. 1959

49 Effort intolerance, dyspnea and light-headedness Right-to-left shunt (over many decades): paradoxical embolus & brain abcess Cause of death: generally unrelated to the congenital malformation Tuchman, H. Am. J. Med. 1956

50 Physical Appearance Normal except for: –Cyanosis –Clubbing

51 Auscultation S 2: single  right ventricular stroke volume and  pulmonary capacitance  early pulmonary valve closure  synchrony or near synchrony with aortic closure Meadows, W. R. Circulation 1961

52 Diagnosis The Echocardiogram 2-D echo + contrast : Gold standard Foale, R. Eur. Heart J. 1983

53 Cyanosis Bluish discoloration of lips, nail beds, ears & malar eminences:  reduced Hb in small blood vessels Central cyanosis: SaO2 < 85 % (Dark-skinned: SaO2 < 75 %) Both mucous membranes & skin Peripheral cyanosis: Spares mucous membranes

54 Clubbing In the 5th Century BC, Hippocrates observed that in empyema “the fingernails become curved and the fingers become warm especially at their tips” The term “Hippocratic fingers” used in early writings Campbell, D. BMJ 1924

55  in thickness of the nail bed and soft tissues of the volar surface followed by connective tissue proliferation, collagen deposition, capillary dilatation, infiltration of lymphocytes and plasma cells Currie, A.E. Chest 1988

56 Mechanism of Clubbing Right-to-left shunt: Megakaryocytes (platelet precursors) bypass pulmonary circulation  systemic circulation  lodge in the tips of the digits (prevailing pattern of blood flow) Dickinson, CJ. Eur. J. Clin. Invest. 1993

57 Once impacted in the capillaries of the digits and periosteum : release: - VEGF (Vascular Endothelial Growth Factor) - Platelet-Derived Growth Factor - Hypoxia-inducible factor-1  - Hypoxia-inducible factor-2  Border, WA. N. Engl. J. Med. 1994

58 Am J Med Sci 2005;329(3): 1- In press


60 “A Tale of 3 Patients…” What have we learned? Don’t trust “Medicine by rumor”, always check for yourself No technology will replace the importance of a good physical exam You can publish publishable cases Learning is an ENDLESS process

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