Case Study:27/07/2012

1. Case Study:27/07/2012 Dr.J.A.Venter

2. Me.E.Pule • UM00586495 • 46 year old G3P3 • Dilated cardiomiopathy on failure therapy. • Diffuse LAP since 4 days prior to admission with no PV bleeding. • LNMP : 4 months ammenoreah • Q – BHCG : 300 IU/L • Sonographichypervascular lesion in a enlarged uterus with gestational trophoblastic disease considered.

3. Me M.E.Pule • Referred to radiology for evaluation of the hypervascular uterine mass by CECT and staging of choriocarcinoma. • CECT – 7/3/2012

4. Diagnosis • Uterine AVM secondary to gestational trophoblastic disease with high – output cardiac failiure.

5. Vascular malformation in the uterus • Originate from congenital or acquired (uterine curettage,caesarean section, gestational trophoblastic neoplasia or endometrial carcinoma ) dysfunctional venous and arterial communications. • Present with heavy vaginal bleeding which does not respond to conventional treatment. • Ultrasound with colour Doppler is the most common initial investigation, with magnetic resonance imaging being used for detailed evaluation. • Angiography remains the gold standard. • Embolization is becoming increasingly the first choice of treatment with excellent results.

6. Vascular malformation in the uterus • Hyper-vascular areas within the uterus : Vascular haemangiomas RPOC Molar pregnancies. • Any uterine hypervascular lesion that does not undergo spontaneous regression in a patient with negative ß-hCG findings is likely to be an AVM

7. Vascular malformation in the uterus • AVMs are errors of morphogenesis with stable cellularity and do not show spontaneous regression. • Haemangiomas are benign neoplasms with increased mitotic activity that undergo a proliferating and involuting phase. • Serum ß-hCG levels can help to exclude RPOC

8. Vascular malformation in the uterus • On MRI, uterine AVMs show up as a focal uterine mass with disruption of the junctional zones, serpinginous flow related signal voids and prominent parametrial vessels • Angiography is the gold standard for definitive diagnosis of AVMs and has the advantage of demonstrating the feeding arteries and draining veins of the AVM

9. Etiology of high-output heart failure • AV fistula (congenital or acquired) • Anemia • Thyrotoxicosis • Cirrhosis • Beriberi • Pregnancy

10. High-output heart failure • The normal range cardiac index 2.5–4.2 L/min/m2. • Patients with cardiac indices below this range have systolic dysfunction. • When the cardiac index is above this range, a patient may develop high-output heart failure as a consequence of supraphysiologic cardiac output due to shunting of blood from the high-pressure arterial side to the low-pressure venous side. This diversion of blood back to the right-sided circulation reduces overall systemic arterial blood flow. The counterregulatory response is an increase in cardiac output mediated by the sympathetic nervous system and circulating catecholamines. • Initially, the heart increases cardiac output via an increase in heart rate and stroke volume. With time, excess cardiac stimulation leads to left ventricular hypertrophy, reduction in LVEF, and eventual heart failure.

11. High-output heart failure • Index of suspicion on the part of the clinician. • Despite their physiologic differences, the signs and symptoms of low-output and high-output heart failure are quite similar. Dyspnea on exertion, fatigue, and fluid retention are the hallmarks of both. • Notable differences in the physical exam may be the presence of a hyperkinetic precordium and wide pulse pressure in the high output state

12. High-output heart failure • Definitive proof a high-output state requires right heart catheterization. High cardiac output, low-normal systemic vascular resistance, and pulmonary hypertension with normal pulmonary vascular resistance are characteristic findings. • Pelvic Angiogram 28/03/2012

13. PCS • Congenital absence of valves. • Nutcracker syndrome - high pressure within the LRV leads to the development of direct communication between the dilated veins and adjacent calyces with consequent hematuria and flank pain. Renocaval pressure gradient > 3 mm Hg. Treated by embolization and renal vein stenting • More frequently on the left than the right • Consequent congestion of the pelvic veins leads to swelling and engorgement that in turn leads to the pain experienced by the patient.

14. Nutcracker Syndrome

15. Clinical Presentation • Post coital pain - hours or days , relieved by lying down. Unilateral or bilateral. Acute and severe ,Chronic and dull - combined with ovarian point tenderness, 94% sensitive and 77% specific for pelvic congestion syndrome. • Bladder irritability ,functional GI symptoms and low back pain. • Depressed and anxious - partly because of the fact that they have very real symptoms for which the cause is often difficult to find

16. Diagnosis • Gonadal venography remains the definitive imaging modality used to evaluate patients with PCS. • Tilting table • Valsalva maneuver using old fashioned sphygmomanometer by asking the patient to blow into the rubber tubing and hold the mercury at approximately 20 mm.

17. PCS Treatment • Hysterectomy and bilateral oopherectomy • Ovarian vein ligation - establishment of collateral channels and recurrence of symptoms. • Endovascular embolization - occlusion of collaterals that drain into the high ovarian vein. Technique is still evolving. • Very important not to build the patients expectations too high as treatment may fail to relieve symptoms.

18. Embolize the incompetent LOV using gelfoam/sclerosent slurry

19. Coil Embolization

20. References • High-output heart failure secondary to arteriovenous fistula Adam B. STERN, Philip J. KLEMMER -Hemodialysis International Volume 15, Issue 1, pages 104–107, January 2011 • Spontaneous Rupture of an Ovarian Artery Aneurysm: Case Report and Review of the Literature Lai-Wan Chao, Chi-Hau Chen Gynecological and Obstetric Investigation Vol. 68, No. 2, 2009 • Vascular malformation in the uterus: a case report and literature review Suman Vohra1, AnthiePapadopoulou and Demetrios L Economides Ultrasound 2011; 19: 102–106. DOI: 10.1258/ult.2011.010056