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From Pregnancy to Pediatrics

From Pregnancy to Pediatrics. Caring for the Fetal Family. NAPNAP WA State Annual Continuing Ed Meeting March 11, 2013. Lani Wolfe, ARNP Seattle Children’s Prenatal Program. Preparing families & health care systems for babies with conditions diagnosed prenatally. PART I.

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From Pregnancy to Pediatrics

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  1. From Pregnancy to Pediatrics • Caring for the Fetal Family NAPNAP WA State Annual Continuing Ed Meeting March 11, 2013 Lani Wolfe, ARNP Seattle Children’s Prenatal Program

  2. Preparing families & health care systems for babies with conditions diagnosed prenatally PART I

  3. The backstory behind the baby you meet…

  4. Prenatal Diagnosis “The goal of prenatal diagnosis is the near-term delivery of a nonhydropic infant whose postnatal management is anticipated” -Cuneo, Bettina 2006. Outcome of fetal cardiac defects. Current Opinion in Pediatrics, 18:490-496

  5. Fetal Care Programs • Program components vary by institution • Seattle Children’s opened in 2007 a Prenatal Diagnosis & Treatment Program • Formalized, thoughtful approach to the needs of families with prenatally diagnosed congenital anomalies

  6. Prenatal Diagnosis & Treatment Program • Accurate diagnosis • Ultrasound • Echocardiogram • MRI • Genetic testing • Counseling- OB + pediatric specialists • Care coordination • Fetal Intervention at Seattle Children’s + partner institutions • Medication for cardiac arrhythmias • Fetal bladder taps/ vesicoamniotic shunts • EXIT procedures for airway management

  7. Prenatally Diagnosed Condition groups (% of prenatal pts at SCH) • Congenital heart disease (60%) - structural - arrhythmias • Neurodevelopmental(15%) - neural tube defects - intracranial anomalies • General surgical conditions (15%) - abdominal wall defects - congenital diaphragmatic hernia - bowel atresias - congenital lung lesions • Urologic/ Nephrologic conditions • Chromosome anomalies • Craniofacial + skeletal

  8. Prenatal Obstetric Care ACOG: American College of Obstetrics & Gynecology • Guidelines for prenatal care: • “Women who receive early and regular prenatal care are more likely to have healthier infants” • specifics of “care” vary • Major birth defects: 2-5% live births • Aim to ID risk factors for poor outcomes “…a significant % of intrapartum & neonatal problems occur among pts without identified antenatal risk factors.” • the case for most of your patients

  9. Prenatal Obstetric Care Antepartum surveillance: recommended for all • HCT/ Hgb, blood group, antibody screen, Rubella immunity, infectious screen, UA, cervical cytology, diabetes screening, CF • If seek care <20wks: offer screening for aneuploidy • Serum screening +/- NT +/- 20w marker US (combined , triple, quad) • Offers composite risk for T-13, T-18, T-21 + ONTD • All women, regardless of age, should have the option of invasive prenatal diagnosis for fetal aneuploidy (CVS or amnio)

  10. Prenatal Obstetric Care Ultrasound: no official standard for low risk pts “…only when there is a valid medical indication for exam.” Typical at major medical and academic centers: • Viability / dating 8-10wks • Anatomy scan at 18-20 wks • Additionally as needed • Size / date mismatch • Abnormal or incomplete initial exam

  11. Influences on Fetal Health • Genetics • Maternal health environment: • Diabetes, Lupus /Sjögren's • Congenital heart disease • Other chronic or heritable conditions • Medications • Pregnancy induced hypertension • Infection • Uterine environment • Amniotic fluid volume, infection, multiple gestation • Placenta & cord • location, quality, cord insertion • A beast of its own– respect the placenta!

  12. Prenatal Counseling Understanding the maternal / familial state: All influence decisions re pregnancy mgmnt & level of neonatal intervention • Age/ stage: teen -- elderly primigravida (AMA) • Profession: health care provider, special ed teacher, military • Planned: ART, last sperm banked FOB w testicular CA • Unplanned: using contraception, post vasectomy or BTL • Geography • regional factors (local communities, mountain passes) • country of origin, resources / societal attitudes

  13. Prenatal Counseling • Cornerstone of the program is unified message and plan between pediatrics & OB: bridging the gap • Preparing the family • Diagnosis • Prognosis • Neonatal – lifespan experience • Multidisciplinary input • Management options available • Emotional support • Logistic planning

  14. Prenatal Counseling  Families are parenting already Walk with families down whatever path they can make most peace with  It’s ok to share hope rather than crush it

  15. Prenatal Care Coordination: Delivery Planning Delivery • Where • When • How • Who • Family logistics

  16. Prenatal Care Coordination • Unique challenges • How to plan for someone who’s not here yet? • No medical record #?? • Origins of fetal care coordination • Ensuring prenatal work is shared in the neonatal period: • provider to provider communication • bridging the gap between obstetrics and pediatrics • SURPRISE!

  17. Prenatal Care Coordination Communication • Stork Report / forecast tool • Weekly teleconference • Cord blood tracking • Pediatric outcomes letters

  18. Stork Report in action!

  19. Goals of Prenatal Diagnosis Improved outcomes: Family logistics Emotional preparedness: families who have already begun the grieving process are better prepared to engage with the baby & care process. Possibly even better aligned as couples Health outcomes Neonatal palliative carefor severe cases DC planning: initiated antenatally

  20. Ethics, Dilemmas, Challenges Dilemmas for families: Pregnancy decision-making (up to 24 wks in WA) Discordant twins Location of care – out of state options Discordant beliefs within the family Full neonatal intervention or comfort care? Inadequate information Limitations of fetal diagnostic technology Encourage families to consider “most likely outcome” in their decision making.

  21. Ethics, Dilemmas, Challenges Dilemmas for providers: • Do we adequately capture the essence? Can we? • Do families have what they need to make their decisions? • Counseling to be or not be neutral? • “what would you do?” • “Parents will always want the right to make decisions but not always the responsibility.” David Woodrum, MD

  22. Part II • Case Studies

  23. Congenital Heart Disease CHD: • Structural CHD = most common congenital anomaly group • ~ 1% of live births are affected • In USA: 32,000/yr births with CHD -- 1400/yr in WAMI region • ↑ risk recurrence to 2-3% after an affected pregnancy • Fetal echocardiogram is mainstay of prenatal diagnosis • As early as 14wks, with completion at > 18wks

  24. Prenatal Diagnosis & Treatment ProgramCHD: fetal echo referrals to PNC  fetal indications  maternal indications Prior child with CHD Maternal CHD Maternal diabetes Maternal Lupus or like condition Exposures: medications, viral illness • Structural anomalies • Chromosomal anomalies • Arrhythmias • Unable to clear heart on routine OB scan • Increased NT • Hydrops

  25. Congenital Heart Disease: HLHS • Several studies comparing the experience of pre / post natal diagnosis of HLHS have found: ♥less preoperative acidosis in the prenatally diagnosed groups ♥ improved survival to surgery ♥no differences in post-operative mortality and survival • study: fewer adverse perioperative neurologic events ♥Rich area of research: long term neurodevelopmental outcomes impact of prenatal diagnosis

  26. Case 1: Hypoplastic Left Heart Syndrome OB History: 32 yo G3 P2→3 1 prior term vaginal delivery + term CS for NRFHT Healthy 14yo & 7yo PMHx: HTN – Rx’d meds x 1yr, dc’d with pregnancy test FMHx: No congenital anomalies Social Hx: Pt & husband active duty military Husband in Afghanistan most of pregnancy

  27. Case 1: Hypoplastic Left Heart Syndrome Prenatal Course Late to care at 18wks – irregular menses, had not realized pregnant Declined serum screening • 20wk anatomy scan: • hypoplastic left heart syndrome • o/w normal fetal growth, amniotic fluid, anatomy Declined amniocentesis

  28. Case 1: Hypoplastic Left Heart Syndrome • Seattle Children’s Prenatal Clinic at 21 +5wks • Fetal echocardiogram confirmed dx: HLHS w mitral + aorticatresia • US: o/w normal fetal growth, amniotic fluid, anatomy • Mildly elevated BP • Thorough counseling: cardiology + MFM combined • Offered range of pregnancy management options • Opted for pregnancy continuation • Joint OB management w local MFM + UW Plan: • serial fetal echo + US f/u for remainder of pregnancy • UW delivery by repeat CS at 39wks • expeditious neonatal transfer to SCH

  29. Hypoplastic Left Heart Syndrome: HLHS • Spectrum of disease, accounts for 1% of CHD • ~20x/yr evaluated at SCH Prenatal Clinic • HLHS + unbalanced AV canal, heterotaxy, HRHS, other single ventricle • Features: • Hypoplastic left heart structures • Cause unknown • Usually tolerated well in utero • Post-natal ↓PVR & ductus closure → ↓systemic cardiac output → shock & metabolic acidosis

  30. Congenital Heart Disease: HLHS Surgical palliation now “standard of care” • Single ventricle surgical pathway • Staged procedures to ultimately separate red / blue circulation • Relies on the single ventricle as systemic pumping chamber • Relies on passive pulmonary blood flow • Systemic venous return bypasses heart, flows passively to lungs • Norwood: 7-10 days (RV-PA shunt, aortic arch reconstruxn, atrial septectomy) • Glenn: 4-6 months (SVC-PA) • Fontan: 3-5 years (IVC-PA)

  31. Congenital Heart Disease: HLHS Counsel: severe, complex structural heart disease • Surgical palliation is not repair • Intense first few yrs: consistent caregiver at home ideal • Morbidity, mortality with surgery + inter- stage • Neurocognitive impacts • Lifelong cardiology f/u • Many do very well, school + activities • ↓ ventricle fxn over time (late teens, early 20’s): transplant Conditions with abnormal lungs or pulmonary vasc may preclude the single ventricle pathway • not a candidate for ultimate Fontan completion • may not be offered a surgical option neonatally

  32. Congenital Heart Disease: HLHS Basic Cardiac Anatomy Hypoplastic Left Heart Syndrome

  33. Fetal Echo Hypoplastic Left Heart: HLHS

  34. Case 1: Hypoplastic Left Heart Syndrome High risk pregnancies + medically complex children take a toll on families. Impact for this dual-military family: • Frequent communication w commanding officers for both parents • Pt single-parented and worked full time until delivery • Required permission (not granted) to wear tennis shoes • Dad returned from Afghanistan 1 week before scheduled CS • Achieved commitment to staying stateside indefinitely thereafter

  35. Case 1: Hypoplastic Left Heart Syndrome Delivery: • Scheduled repeat CS at 39wks at UW Newborn: • BW 3129g • Apgars 3 (1min) 6 (5min) • Limp, poor resp effort, low 02 sats • Stim, PPV, CPAP • PGE infusion • Transferred to SCH within 4hrs

  36. Case 1: Hypoplastic Left Heart Syndrome • 4 days: OR modified Norwood w Sano shunt • 10days: OR delayed sternal closure • 29days: DC’d home on nc oxygen, NG feeds + sat monitor • Frequent cardiology + PCP f/u • 5 months: Glenn + patch repair of PAs+aaorta • Post-procedure cardiac arrest, CPR, prolonged intubation in CICU • Cath – stents placed in LPA + portion of aorta • Hospitalized nearly 2 months (pulm HTN, opiate wean) • Readmitted x1 for diuretic adjustment • Borderline decreased RV function

  37. Case 2: Diaphragmatic Hernia OB History Healthy 34 yo G2 P1→2 Soc: married, planned pregnancy, healthy 2yo sibling FMHx FOB’s younger brother - congenital diaphragmatic hernia - died at 1 wk of life, no other anomalies FOB’s older brother - neonatal death, etiology unknown

  38. Case 2: Diaphragmatic Hernia Prenatal Course • Declined prenatal serum screening • ~20wks anatomy scan: left diaphragmatic hernia • Stomach ↑ in left chest • Liver ↓ in abdomen • LHR 1.1 • Heart shifted right • High normal amniotic fluid • o/w normal fetal growth + anatomy • Amniocentesis: normal karyotype, 46XY, declined microarray

  39. Case 2: Diaphragmatic Hernia 24 wk US stomach & heart at same level

  40. Case 2: Diaphragmatic Hernia • CDH: • incomplete devpt of diaphragm muscle • abdominal contents herniate into chest • Occurs early (4-8wks GA), during formation of diaphragm • 1:4000 live births (1:3000 pregnancy, IUFD, TOP) • Etiology unknown • Pulm hypoplasia + pulm HTN are hallmarks • 80% L sided, prognosis worse for R sided • 15-25% have associated anomalies

  41. Case 2: Diaphragmatic Hernia • CDH prenatal detection rate: ~60% • Predictors of outcome: survival & need for ECMO • Liver position is most important prognostic factor across studies • + lung volume estimates • Liver ↓ + LHR >1.4 = most favorable outcomes (~25% cases) • Liver ↑ + LHR <1.0 = worst outcomes (~25% cases) • Predictors of lung volume • Lung to Head Ratio (LHR) by US • MRI – institution dependent expertise • 3D US

  42. Case 2: Diaphragmatic Hernia • UCSF Fetal Treatment Ctr at 23 + 3wks • Liver ↑ in chest • LHR 0.9 • Mild polyhydramnios (AFI 22.9cm) • Normal fetal echo • Normal growth, no other structural anomalies • fetal tracheal occlusion was offered Fetal intervention criteria at UCSF: • LHR < 1 (+ liver up) • Normal karyotype • <28 wks • No other anomalies or maternal contraindications

  43. Case 2: Diaphragmatic Hernia Seattle Children’s Prenatal Clinic • Consult pediatric surgeon + fetal echocardiogram Close OB monitoring • LHR stable, improved 1.2-1.4 • steadily increasing AFI – up to 50cm (double normal) • amnioreduction offered Declined offer for tracheal occlusion at UCSF Family Preparation: • 2 hospital tours: included older sibling • CDH support group + linked w families thru Parent Support Program • Neonatology + social work consultations

  44. Case 2: Diaphragmatic Hernia • Newborn Course • 38+3wk delivery by scheduled C/S, BW 3.3 kg • High-frequency oscillator • Transferred to SCH at 3hrs of life

  45. Case 2: Diaphragmatic Hernia • Worsening blood gases + pulmonary HTN • Oscillator + inhaled Nitrous Oxide • ECMO on DOL #3 x 14 days Patch repair of “extremely large” CHD on DOL # 23 • “diaphragm was essentially almost nonexistent” • Gore-Tex + Marlex patch • Left lobe of the liver + stomach + bowel in left chest

  46. Case 2: Diaphragmatic Hernia – first + last day of ECMO

  47. Case 2: Diaphragmatic Hernia

  48. Case 2: Diaphragmatic Hernia: pre-op + post-op films

  49. Case 2: Diaphragmatic Hernia Post-Op Course: • POD # 16 Extubated to CPAP, then to nasal cannula • Age 7.5wks: off TPN, full enteral feeds, fortified BM via ND tube • 2 months (62days)- DC’d home • Oxygen by nasal cannula (0.2 Lpm) • NG feeds + small volume PO training • Meds: oral sildenafil, PPI, Reglan, clonidine Age 1 yr: • 9mo- Pulm HTN resolved, O2+ Sildenafil DC’d • All PO, growing & developing well, active • No significant respiratory illnesses • No re-admissions to the hospital • Mild URIs have not required oxygen since it was DC’d

  50. Case 2: Diaphragmatic Hernia 2yr f/u: • Off O2 • Off all meds • Gen Surg: • “looks absolutely fantastic!” (surgeon) • Pulm: bronchitis x2, albuterol w URIs, sats 100% RA • Cardiology: nml, pulm HTN resolved • Orthopedics: no apparent scoliosis, annual f/u • Audiology: nml hearing, f/u 6 mo • Devpt: nml growth, devpt, energy, activity w peers/sib, lang/ cognitive • Genetics: abnormalmicroarray pt + FOB + FOB’s dad, sib -

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