Case Report. Anaesthetic Management for Birth in the presence of a rare form of Congenital Heart Disease. Dr. Moritz Schürch

Transcription

1 Case Report Anaesthetic Management for Birth in the presence of a rare form of Congenital Heart Disease Dr. Moritz Schürch

2 The Question anaesthetic consultation: assessment and management plan for birth 22 yo 1GOP; 34 weeks of gestation Congenital Heart Disease: Shone s Syndrom

3 The Question anaesthetic consultation: assessment and management plan for birth 22 yr old 1GOP; 34 weeks of gestation Congenital Heart Disease: Shone s Syndrom bicuspid aortic valve with combined lesion coarctation repair 9/88 und 6/95 reconstruction of aortic valve, repair of subaortic stenosis and ventriculotomy 10/92 recurrent moderate coarctation: p-mean 32 mmhg

4 The Cardiologists Opinion Stable situation Coarctation still moderate No objection against vaginal delivery Delivery in a hospital with cardiologist If cesarean delivery is required they recommend general anaesthesia peripheral vasoplegia under spinal anaesthesie with danger of poststenotic hypotension and compromised uterine perfusion

5 Outline What is Shone s Syndrom? Information in textbooks and guidelines? Information in published papers? What shall we do in the actual case? Discussion

6 Shone s Syndrom First Publication:

7 Shone s Syndrom Morphogenesis The mitral valve complex and the left ventricular outflow tract are an entity, forming the medial and superior portion of the ventricle Congenital malformations of the mitral valve can affect the anatomy of the subaortic region One abnormality upstream can during morphogenesis result in distal defects due to abnormalities in flows

8 Shone s Syndrom Supravalvular Ring of left Atrium circumferential fibrous ring hypoplastic mitral valve +/- malformation of the leaflets Parachute Mitral Valve missing anterior papillary muscle, chordae converge onto one large papillary muscle, parachute like appearance Subaortic Stenosis Coarctation of the Aorta usually peri- or postductal 0.6% of all cases of CHD, : = 1.5 : 1

9 Shone s in TEE ME-4Ch dtg-lax ME-lax Ray Liao, Washington UE-aortic-arch-lax

10 Shone s Syndrom: Correction Sekhar, Indian J. Anaesth 2004

11 Our Patient 22 yr old 1GOP; 34th week of gestation Congenital Heart Disease: Shone s Syndrom bicuspid aortic valve with combined lesion coarctation repair 9/88 und 6/95 reconstruction of aortic valve, repair of subaortic stenosis and ventriculotomy 10/92 recurrent moderate coarctation: p-mean 32 mmhg

12 How shall we proceed? Vaginal Delivery? with Option of Epidural? without Option of Epidural? Plan B in case of need for cesarean section? Elective Cesaren Section? With spinal anaesthesia? With epidural anaesthesia? With general Anaesthesia? Transfer to St. Elsewhere-Hospital? Other suggestions?

13 How shall we proceed? Vaginal Delivery? with option of epidural analgesia? without option of epidural analgesia? Plan B in case of need for cesarean section? Elective Cesarean Section? With spinal anaesthesia? With epidural anaesthesia? With general Anaesthesia? Transfer to St. Elsewhere-Hospital? Other suggestions?

14 An exposed path with uncertain exit?

15 That s what Textbooks and Guidelines say

16 The Textbooks No concrete information about Shone s Syndrom Aortic Stenosis and Coarctation of the Aorta: Vaginal Delivery: systemic medication, inhalational analgesia, pudendal nerve block AS: extreme caution with techniques that reduce systemic vascular resistance and venous return Cesarean section: light general anaesthesia, maintain increased level of heart rate, contractility and vascular resistance

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18 Risk in Pregnancy and (C)HD cardiac dis. in preg. Tutorial 1&2 Reide, Yentis; j.bpobgyn 2010 Dob, Yentis; Int J Obstet Anaesth. 2006

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22 That s what published papers say

23 Shone s und Anästhesie

24 Shone s, SS und Anästhesie

25 Shone s und SS 15 yr old 1G 0P 25 weeks of gestation Dyspnea on exertion since age of 9, increasing during pregnancy, flue like symptoms since one week BP 150/98 mmhg, Hf 102/min, RF 22 Rx: pulmonary edema TTE: - severe MS, supravalv. Ring, p-mean 22mmHg - 2 papillary muscels, MV: no parachute-configuration - bicucpide AV, Coarctation with p 70mmHg Dg: Shone-Syndrom

26 Shone s und SS Therapy Bed rest, treatment with Furosemide and -blocker Transfusion of 2 packed RBCs at Hb 10.9 g/dl Reduction von PAP sys from 70 to 50 mmhg Reduction p-mean over MV from 22 to 14 mmhg Induction of labour at 37 weeks with radial arterial line, hemodynamically stable, uncomplicated delivery Discharge 4 days after delivery partum with follow up by cardiologist

27 Shone s und SS Discussion Hemodynamic changes during pregnancy can cause decompensation of CHD It is not coincidental that the patient presented at 25 weeks gestation, since this is the time in pregnancy when cardiac output is maximal Cardiac decompensation during pregnancy needs careful evaluation Surgical correction during pregnancy would most likely result in a high fetal mortality rate

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29 Shone s und Sectio 17 yr old 1G 0P 39 weeks of gestation Shone s Syndrom, parachute MV with mild stenoses, bicuspide AV, mild subaortic stenosis Followed in congenital heart clinic since 2 year old. At 14 yrs fatigue with exercise, mild MS and AS, no intervention At 16 yrs pediatric cardiology clinic: 16 wk gestation at 26 wk: p-mean MV 6mmHg, p-mean suba 36mmHg At 36 wk: decision cesarean section based upon multiple level heart obstruction and symptoms

30 Shone s und Sectio Cesarean Delivery at 39 weeks of gestation BP 103/51 mmhg, Hf 93/min, SpO 2 (Air) 98% EKG, NIBP (1x/min), SpO 2, O 2 nasal, 500ml RL EDA (L3/4, si. med.), Lido 2% +Adr. 2x1.5ml / 3ml BP-drop to 82/30mmHg, 54/30mmHg despite Phenylephrine, Ephedrine, RL. FHR 60/min after normalising BP; immediate delivery Inadequate Block; GA RSI (Pento 100mg, Succi) Healthy infant, APGAR 9/9, ph 7.13

31 Shone s und Sectio Discussion Optimal anesthetic technique for cesarean delivery in patients with stenotic valvular lesions is controversial Etiology of the rapid and profound hypotension after 6ml of epidural Lidocaine is unclear initiate invasive monitoring before the initiation of anesthesia patients should be managed by a multidisciplinary team with a management plan regarding the timing and mode of delivery

32 Coarctation and Cesarean Section T. Togal (EJA 2002; 19:760-73) 20 yr old patient 40 weeks of gestation BD 230/97mmHg re; 107/67 mmhg li Arm diminished lower extremity pulses Echo: 50% narrowing of aortic isthmus, LVEF 67% Fetal distress demands urgent Cesarean Section right radial arterial and internal jugular catheter; GA with RSI; Etomidate, Alfentanil, Succinylcholin, Vecuronium, Sevoflurane, N2O/O2 Avoidance of sympathetic block that occurs with RA. Risk of significant hypotension (blood flow to fetus!)

33 Coarctatio und SS

34 Coarctatio und SS

35 Back to our Question What should we recommend?

36 How shall we proceed? Vaginal Delivery? with option of epidural analgesia? without option of epidural analgesia? Plan B in case of need for cesarean section? Elective Cesarean Section? With spinal anaesthesia? With epidural anaesthesia? With general Anaesthesia? Transfer to St. Elsewhere-Hospital? Other suggestions?

37 Our Consideration Avoidance of neuraxial Block Effect on poststenotic Blood Pressure with compormised uteroplacentar perfusion? Avoidance of urgent Cesarean Delivery Availability of experienced anaesthesiologist and cardiologist! Reduction of Risk with planned Cesarean Section? Availability of anaesthesiologist and cardiologist invasive monitoring, availability of TEE ideal: procedure in central operation tract

38 X

39 Implementation Planned CS 38 5 / 7 weeks of gestation ECG, NIBP (li), SpO2, peripheral Line right A. brachialis cath. (A.rad. Ø US-signal) NIBPleft 95/52mmHg, IBPright 142/92mmHg CO-monitoring (LiDCO Rapid), TEE ready GA RSI Thiopental (350mg), Fentanyl (0.1mg), Succinylcholin (60mg), N2O/O2, Isoflurane After cord clamping: Clamoxyl 1g, Syntocinon 5 E (slow infusion), Change to Propofol Ephedrine S 10mg, Phenylephrine S 0.15mg Blood loss 650ml, RL 900ml

40 Hämodynamics Induction 4 Ephedrin 10mg 2 Intubation, Knife to Skin Phenylephrin 0.15mg 3 Syntocinon 5 IE KI 5 Extubation

41 TTE

42 Postoperative Course Transfer to Intensive Care Unit Extubatet immediately after surgery Hemodynamically stable Uneventful postoperative Course Transfer to puerperal ward on the same day Uneventful post-partum period Discharged home on the 5 th postoperative day with a healthy baby

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46 ? ; urgent CS 37 yr old 1G 0P 40 3 / 7 weeks of gestation Amniotic infection syndrome, mildly preeclamptic Loud systolic murmur Scar from Sternotomy; Correction of congenital heart disease at the age or 4 No documentation at all! Narrowing of one of the arteries, little whole at the other artery!

47 Discussion? Thank you!