Veni, vidi, vici I came, I saw, I... Conquered? Discussion and cases from my MFM rotation. Perinatal rounds March 30 th, 2010 Momoe Hyakutake, PGY 3

Transcription

1 Veni, vidi, vici I came, I saw, I.... Conquered? Discussion and cases from my MFM rotation Perinatal rounds March 30 th, 2010 Momoe Hyakutake, PGY 3

2 Case based discussion on the following: 1) CCAM 2) Fetal tachycardia 3) Indomethacin tocolysis and ductus arteriosus constriction

3 Case 1

4 Mrs. TB 20 years old G2P1 LMP September 16, 2009 EDC June 26, 2010 based on LMP Regular 30 day cycles Sure of dates Routine prenatal care starting at 10 weeks GA

5 Mrs. TB Past medical history None Past surgical history None Past obstetrical history G1 2008, SVD of male infant at term, 8lbs Past gynecological history unremarkable

6 Mrs. TB Medications Prenatal vitamins Not taken preconception Allergies None Social history Quit smoking in 2009 Denies alcohol use Denies recreational drug use Family history unremarkable

7 Mrs. TB Abdominal pain at 9 weeks GA Normal ultrasound 9 weeks 2 days Adjusted EDC July 5, 2010 Pain subsequently resolved spontaneously MSS offered Patient declined Routine ultrasound on February 18, weeks GA Abnormal findings..... URGENT referral to perinatal!!!!!

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10 CCAM Congenital cystic adenomatoid malformation of the lung Also called CPAM Developmental anomaly of the respiratory tract Excessive proliferation of terminal bronchioles, lacks normal alveoli Most common congenital lung lesion Incidence of 1:35,000

11 CCAM Can occur in either lung with no predominance Slightly higher incidence in males than females Mostly unilobar (85-95%)

12 Type % of postnatal cases Single or multiple large cysts surrounded by smaller cysts and compressed normal parenchyma Large cysts lined with pseudostratified ciliated epithelium Smaller cysts lined by cuboidal to columnar epithelium Associated anomalies rarely seen

13 Type % of postnatal cases Numerous smaller cysts Lined by cuboidal to columnar epithelial cells with thin, underlying fibromuscular layer Assiciated with higher incidence of cardiac, renal and chromosomal anomalies

14 Type % of postnatal cases Male predominance Small cystic lesions or solid lesions Adenomatoid in appearance

15 Type 0 Acinar dysplasia or agenesis Rare Incompatible with life

16 Type % of cases Peripheral cyst type Hamartomatous malformation of distal acinus Large thin-walled cysts located at periphery of the lobe, lined by smooth membrane

17 CCAM vs. BPS CCAM Communicates with the tracheobronchial tree Derives their arterial blood supply and venous drainage from normal pulmonary circulation BPS No connection to tracheobronchial tree Derives blood supply from anomalous systemic artery

18 Natural history Unpredictable growth between weeks GA Up to 40% undergo rapid proliferation and tumor enlargement Regression in 15% (rare if hydrops has developed) Growth usually reaches a plateau at 28 weeks

19 Adzick et al. 1998

20 Prognostic factors CCAM volume to head circumference ratio (CVR) height x width x length x 0.52 head circumference CVR 1.6, 80% risk of hydrops CVR 1.6, 2% risk of hydrops ***CCAMs with a dominant cyst may be unpredictable in their growth and expansion so should be excluded from this calculation

21 CVR = 4.7 x 2.8 x 4.3 x 0.52 = Therefore less than 2% risk of developing fetal hydrops

22 Treatment options Expectant Thoracocentesis / cyst-amniotic shunt Macrocystic, unilocular Percutaneous Fetal sclerotherapy Antenatal corticosteriod administration In utero fetal lobectomy Ex utero intrapartum therapy

23 Antenatal steriod treatment Curran et al Journal of pediatric surgery, 2010 Retrospective review 16 patients with predominantly microcystic CCAMs treated with single course of steriod 3 excluded, loss to follow-up 13/13 survived to delivery, 11/13 (85%) survived to neonatal discharge

24 Antenatal steriod treatment Morris et al. Journal of pediatric surgery, 2009 Retrospective review 15 patients with CCAM 13 hydropic, 2 non hydropic 7/13 (54%) initially responded to steroids 8/15 survived neonatal discharge Survival rate 53%

25 Antenatal steriod treatment Peranteau et al. Fetal diagnosis and therapy, 2007 Retrospective review 11 patients (10 micro, 1 macro) 5 patients had hydrops 4/5 (80%) resolution of hydrops 100% survival in all previously hydropic fetuses

26 Summery CCAM is the most common congenital lung lesion In the presence of hydrops mortality is 100% CVR can help identify fetuses at risk of developing hydrops Choice of therapy depends on type of lesion (microcystic vs macrocystic) Promising evidence supporting the use of antenatal steriods in the treatment of CCAM, espcially when its microcystic

27 Case 2

28 Mrs. NZ 29 years old G1P0 Started seeing her obstetrical care provider at 5 weeks gestation

29 Mrs. NZ LMP July 27, 2009 Regular monthly cycles EDD May 4, 2010 Past medical history Hypothyroidism Medications Synthroid 0.1mg po daily Folic acid 1mg po daily Allergy Lactose

30 Mrs. NZ Past surgical history Appendectomy Family history Niece with an atrial septal defect (no repair) Social history Non smoker No alcohol No recreational drug use

31 Mrs. NZ Uneventful first trimester Seen regularly for prenatal care Prenatal visit at 28+3 weeks GA.....

32 Fetal tachyarrhythmia

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35 Work up Detailed ultrasounds evaluation of anatomy Cardiac Signs of hydrops Amniotic fluid level growth Fetal echocardiography Identify atrial and corresponding ventricular activity Cardiac function M mode Doppler

36 M-Mode echocardiography Simultaneous trace of ventricular and atrial contraction Cardiac rhythm and rate

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38 Pulse wave doppler echocardiography Transducer transmits a series of pulses to detect motion Doppler shift that is measured is processed and then shown on screen in a spectral display Flow direction Flow velocity Flow characteristics

39 Pulse wave doppler echocardiography To assess cardiac arrhythmia Superior vena cava / ascending aorta Pulmonary vein / pulmonary artery Left ventricular inflow / outflow Innominate vein / aortic arch

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48 Observation Management of fetal tachycardia No therapy but fetus is regularly monitored Delivery Prenatal therapy Transplacental drug therapy Direct fetal therapy Umbilical vein, IM, intraperitoneal, intraamniotic, or combination

49 Benign Premature atrial and ventricular No treatment required contractions Sustained tachyarrhythmia may develop in up to 1% of fetuses

50 Supraventricular tachycardia Deliver for neonatal medical treatment if term In utero medical treatment Can convert to normal sinus rhythm within 48 hours Conversion occurs easier in absence of hydrops

51 Digoxin Prolongs refractory period of the AV node Positive inotropic and negative chronotropic properties Placental transfer Fetal : maternal plasma concentration ratios between 0.4 and 0.9 Significantly decreased with hydrops

52 Digoxin Maternal adverse effects Anorexia Nausea Vomiting Headache Arrhythmia (ventricular extrasystoles, heart block) Contraindicated in Wolff-Parkinson-White syndrome

53 Digoxin Fetal tachycardia without hydrops Conversion rates of 32 71% Fetal tachycardia with hydrops Conversion rates of 10 20% (transplacental)

54 Sotalol Non selective β-blocker Class III antiarrhythmic Prolongs duration of action potential Increases refractory period of atrial and ventricular tissue Inhibits conduction in accessory bypass tracts Placental transfer Fetal : maternal plasma concentration ratios between 0.47 to 1.42

55 Sotalol Maternal adverse effects Fatigue Dizziness Dyspnea Palpattions Nausea / vomiting Torsade de Pointes tachycardia, 2.4%

56 Summery Sustained tachycardia can cause heart failure and lead to hydrops fetalis Fetal echocardiogram is an essential imaging technique to determine the type of tachycardia Both digoxin and sotalol are good first line treatment options If medications are started, must watch the patient carefully for side effects

57 Case 3

58 Mrs. BF 31 year old G2P1 at 30+2 weeks GA Presents to rural hospital with complaints of abdominal pain Started at 18:00, q10 minutes Increasing in frequency and intensity Seen by MD at 21:00 On exam, cervix is long, closed and posterior, soft.

59 Mrs. BF EDC April 12, 2010 based on 20 week ultrasound OB history 2009 delivery of female 34 weeks GA by cesarean section secondary to preeclampsia Current pregnancy minimal prenatal care. Normal 20 week ultrasound. Normal BP Pmhx Mild asthma

60 Mrs. BF Medications None Allergies No known drug allergies Social history Non smoker Denies alcohol use Denies recreational drug use

61 Mrs. BF 22:20 Patient given indomethacin 100mg PR 22:35 Patient given dexamethasone 6mg IM Transfer to RAH

62 Indomethacine Prostaglandin synthetase inhibitor First reported use in 1974 Nonselective inhibition of enzyme COX

63 PGE 2 and PGF 2 Increase myometrial contractility Coordinate contractions

64 Cochrane review, 2005 Compared to placebo Reduction in birth before 37 weeks (RR 0.21) Increase in gestation age, weighted mean difference 3.53 weeks Increase in birth weight, weighted mean difference g Compared to other tocolytics Reduction in birth before 37 weeks (RR.53)

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71 Moise et al. 1993

72 Vermillion et al Retrospective review 72 fetuses 50% developed ductal constriction Mean age 30.9 ± 2.3 weeks Ductal constriction occurred by 31 weeks GA in 70% of affected fetuses

73 Vermillion et al. 1997

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75 Should a different tocolytic been used?

76 Magnesium sulfate Most frequently used tocolytic in the United states Extracellular magnesium Suppresses calcium influx across cell membrane Intracellular magnesium Competitive inhibitor Inhibits myosin light-chain kinase activity

77 Magnesium sulfate Maternal side effects Nausea Vomiting Flushing Lethargy Dizziness Blurry vision Headache Pulmonary edema Shortness of breath Chest pain Cardiac arrest Pulmonary edema Shortness of breath Chest pain Cardiac arrest

78 Magnesium sulfate Cochrane review, 2002 No evidence of a clinically important tocolytic effect No evidence of any substantial improvements in neonatal morbidity Associated with an increase in fetal and pediatric deaths

79 Nifedipine Calcium channel blocker Inhibits calcium re-uptake by voltage dependent calcium channels in the myometrial cell wall Results in relaxation

80 Nifedipine Cochrane review 2002 Compared to any other tocolytic agents Statistically significant decrease in number of women giving birth within 7days (RR 0.76, 95% CI 0.60 to 0.97) and prior to 34 weeks GA (RR 0.83, 95% CI 0.69 to 0.99) NNT 11 (95% CI 6 to 100) Less respiratory distress syndrome, intraventricular hemorrhage, necrotising enterocolitis, jaundice Marked reduction in adverse maternal side effects

81 Summery Use of indomethacin between weeks GA should be carefully considered Consider ultrasound study of the ductus arteriosus if indomethacin is given after 30 weeks GA Nifedipine is a good alternative to indomethacin