Presented by: Lynette Barnhart RNC, BSN, SNNP
June 9, 2014
University of Texas Medical Branch at Galveston
NNP Concepts and Practicum II
GNRS 5632
Dr. Debra Armentrout PhD, RN, MSN, NNP-BC
Dr. Leigh Ann Cates PhD, APRN, NNP-BC,RRT-NPS,CHSE
Maternal History
Maternal and Fetal Risks and Complications
Delivery and Stabilization
Admission exam and diagnostics
Primary admission diagnoses
Etiology and Pathophysiology of Primary Admission Diagnoses
Initial Plan of Care
Hospital Course by Symptoms
Medications
Pertinent Theories and Evidence Based Practice
Family Interactions
Discharge Plan and Follow Up
37 yo, married Hispanic
G6 now P5
1 SAB
1994-39 weeks
1997-40 weeks
2004-39 weeks
2009-39 weeks
EDD 6/10/14
A+, GBS-, RPR NR, Rubella Immune, HIV NR, HBUnremarkable Medical history
Denies alcohol, tobacco, and illicit drug use
Advanced maternal age
37 year old
U/S 12/16/14
Nuchal thickness of 3.1
Low AFP 12/31/14
+ Trisomy 21
U/S 2/28/14
Suspected Coarctation of the aorta
Scheduled induction at 39 weeks
Due to suspected Coarctation of the aorta
Spontaneous labor
38 1/7 weeks GA
SROM 2 ½ hours prior to delivery
Clear fluid
Vertex presentation
NSVD
No maternal medications prior to delivery
Apgars
8 at 1 minute
8 at 5 minutes
Delivery summary
Lusty cry at delivery, cord clamped and cut. Infant taken to radiant warmer. Dried
and stimulated. Infant dusky, BBO2 (40%) provided at 5 minutes of life for preductal saturations below 85%. Continued to dry and stimulate. Improvement in
color and saturations noted. O2 discontinued at 8 minutes of life. Infant placed
skin to skin with mother prior to transfer to NICU for transition.
Gestational age
38 1/7 weeks
Measurements
Birth weight: 3078gms (10th-50th %tile)
Length: 48.5 cm (10th-50th %tile)
OFC: 32 cm (10th %tile)
Vital signs
Temperature 36.9 C
Heart rate 120 bpm
Respiratory rate 40 br/min
Pre-ductal SpO2 94%
Post-ductal SpO2 89%
Physical Exam
General: Habitus of Trisomy 21, No acute distress, active with exam, sucking on pacifier
Eye: Normal conjunctiva, bilateral red reflexes.
HENT: Normocephalic, nares patent, oral mucosa moist with intact palate, anterior
fontanelle soft and flat, ears normally set and rotated, flat facial profile.
Respiratory: Unlabored respirations with appropriate chest excursion, lung sounds
clear and equal bilaterally.
Cardiovascular: Heart rate and rhythm regular without murmur, pulses equal in all four
extremities, capillary refill less than 3 seconds, pink with acrocyanosis to hands and
feet.
Gastrointestinal: Soft, non-tender, non distended with active bowel sounds, no
organomegaly, 3 vessel umbilical cord, anus appears patent.
Genitourinary: Normal male genitalia for age/gestation, testes descended, void at
delivery
Lymphatic: Fat pad to nape of neck
Musculoskeletal: Moves all extremities, no hip clicks, normal Barlow’s and Ortolani’s.
Simian creases to both hands, wide gap between first and second toes.
Integumentary: Warm, dry, pink, without rash
Neurologic: Alert, moves all extremities appropriately.
Echocardiogram
5/28/2014- 1 hour of life
FISH chromosomes
5/29/2014- results pending
High resolution chromosomes
5/29/2014- results pending
Bedside chemstrip
51 @ 1426 (admit)
38.1 week AGA male infant, 3078gms
Suspected Coarctation of the aorta
Suspected Trisomy 21
Narrowing of the aorta
Constricted segment of the
aorta that comprises localized
medial thickening.
5-8% of all congenital heart
defects
May also occur with bicuspid
aortic valve and VSD
(Cates, 2009)
Increased afterload on the left ventricle
Increased wall stress resulting in ventricular
hypertrophy
As the PDA closes (may cause rapid CHF and shock)
Causes increased left ventricular pressures, increased left
atrial pressure, and opening of the foramen ovale
Resulting in left-to-right shunting and dilation of the right
atrium and right ventricle
When the foramen ovale doesn’t open the pulmonary
venous and artery pressures increase
Resulting in right ventricular dilation
(Gholampour, et al., 2006)
Clinical findings
Systolic ejection murmur that radiates to the apex and is often loudest
in the back
Xray finidngs
Generalized cardiomegaly with normal pulmonary vascular density
(Pre-ductal Coarctation)
Enlarges left ventricle and left atrium and dilated ascending aorta
(Post-ductal Coarctation)
Pre-ductal and post-ductal SpO2 saturation discrepancy
Upper and lower extremity BP discrepancy
Absent or decreased lower extremity pulses
Tachypnea
Lethargy
Poor feeding
Shock
(Gomella, 2013)
Cyanotic lower extremities
(Syamasundar, 2012)
Medical Treatment
Prostaglandin E
0.05-0.15 mcg/kg/min
Ventilatory assistance
To support increased work of breathing
Inotropic drugs
Dopamine, Dobutamine, Epinepherine
Diuretics
Used for infants with CHF
Foley Catheter
Strict monitoring of urine output and renal perfusion
UAC
ABGs
(Cates, 2009)
Surgical Treatment
Goal is to bypass the narrowed section of the aorta
Left subclavian aortoplasty
Resection and end-to-end anastomosis
Angioplasty with Dacron patch
Stenting
Balloon angioplasty
(Gholampour, et al, 2004).
Down Syndrome
Presence of an extra 21st chromosome
Most common of all multiple congenital anomaly
(MCA) syndromes
Occurs in ~ 1 in 650 births
Neonatal mortality is related to severe cardiac
anomalies
Maternal nondisjunction
Occurring in the first meiotic division accounts for ~65% of cases
Occurring in the second meiotic division accounts for ~23% of
cases
(Blackburn, 2007)
(Gomella, 2013)
1st trimester screening
Ultrasound between 10 4/7 and 13 4/7 weeks gestation
Measurement of the nuchal translucency (increased in Trisomy 21)
Detects 70% of Trisomy 21
2nd trimester screening
Serum screening between 15 and 20 6/7 weeks gestation
Alpha fetoprotein (AFP), hCG, and unconjugated estriol (Triple
Screen)
Alpha fetoprotein (AFP), hCG, unconjugated estriol, and inhibin-A
(Quad screen) results in 80% detection
Decreased AFP, increased hCG, decreased PAPP-A, and increased
Inhibin A
(Bajaj & Gross, 2011)
Physical findings
Hypotonia, poor or absent moro reflex, flat
facial profile, protruding tongue, up slanting
palperbral fissures, Brushfield spots, anomalous
auricles, joint hyperextensibility, excess nuchal
skin, fifth-digit brachydactyly/clinodactyly, and
single transverse palmar crease.
Associated Anomalies
Congenital heart defects in 40% of infants (most
common ASD or VSD), PDA, endocardial cushion
defect, aberrant subclavian artery.
Hirschsprung disease, duodenal or esphageal
atresia, imperforate anus, hearing loss (66%),
mental deficiency, and renal and urinary tract
(Gomella, 2013)
anomalies.
(Jones, 2006)
Treatment
There is no cure for Down’s
syndrome
Supportive care
Early intervention is essential to assist
in the progression of developmental
milestones.
(Heyn & Perlstein, 2014)
Admit to NICU, place on continuous cardio/resp monitoring
Daily weight, OFC and Length Q Sunday
Erythromycin ointment to eyes bilaterally within 1 hour of
birth
Vitamin K 1mg IM x1 within 1 hour of birth
Place PIV
Infuse D10W at 10cc/hr (80ml/kg/day)
Strict Intake and Output
Cardiac ECHO stat to evaluate fetal diagnosis of suspected
Coarctation of the Aorta
Pre and post-ductal SpO2 monitoring
Obtain 4 point Blood pressures
Chemstick on admission and then per policy
Obtain Metabolic Screen at 24-48 hours
Obtain Metabolic screen #2 at 5-10 days of age
May breast feed ad lib on demand
Social service consult
Respiratory: Mild respiratory insufficiency
At 3 hours of life infant noted to have frequent desaturations (75-85%)
Nasal Cannula started at 1 L @ 21%
Discontinued on DOL 1 (24 hours after start)
Intermittent nasal stuffiness without increase work of breathing
On DOL 4 Treated with little noses Q 4 hours x 24 hours with some
improvement
No further intervention required
Discharged on room air on DOL 6
Pre and post ductal saturation monitoring continued until discharge
Respiratory rate 23-77 during stay
Cardiovascular: Suspected Coarctation of the Aorta
Hemodynamically stable without murmur. Peripheral pulses equal x 4
DOL 0 @ 1 hour of life ECHO obtained
Study limited by technical limitations. Atrial level shunt, quantitatively normal
biventricular size and systolic function. Ventricular level shunting cannot be
excluded. Greater than ½ systemic right ventricular systolic pressures. Tapering of
the transverse aorta without evidence of discrete narrowing or stenosis; coarctation
cannot be excluded in the presence of a ductus.
DOL 1
Pre ductal sats 94-99%
Post ductal sats 94-97%
Left upper extremity BP 81/45 (58)
Left lower extremity BP 70/41 (51)
Heart rate 108-138 bpm
Cardiovascular continued
DOL 2 repeat ECHO
Small superior atrial level communication with left-to-right flow. Mild to moderate
right heart enlargement as well as right ventricular hypertrophy with normal right
ventricular systolic function. Structurally normal-appearing mitral valve with mild
mitral regurgitation. Normal left ventricular size and systolic function in the
presence of flattened interventricular septal wall motion. Moderate sized patent
ductus arteriosus with bidirectional flow. This is a left arch with a common
brachiocephalic trunk as the first arch vessel. The transverse aortic arch is diffusely
narrowed measuring approximately 3.5mmfor a Z-score of negative 2.5. There is
mildly turbulent color low through the transverse aortic arch and there is a peak
gradient of 8mmHg in the descending aorta. There does not appear to be a further
discrete narrowing of the aortic isthmus. However, cannot completely rule out
coarctation in the presence of a PDA. Normal left coronary artery origin. Right
coronary artery origin not well visualized. Systemic to supersystemic RV pressure by
septal position, TR jet, and PDA flow.
DOL 6
Discharge home with parents.
Follow up with Cardiologist in 1-2 weeks.
FEN/GI:
DOL 0
PIV with D10W @ 80ml/kg/day
DOL 1
PIV continues @ 80ml/kg/day
BF or Similac Advance 20 Infant directed Q 2-4 hours
DOL 2
PIV at 117ml/kg/day
BF or Similac Advance 20 15cc Q 3 hours Nipple or gavage
DOL 3
PIV at 125ml/kg/day
BF of Similac Advance 20 15cc Q 3 hours
DOL 4
PIV D10W with lytes at 125ml/kg/day
BF/EBM/Similac Advance 20 Ad lib minimum 15cc Q 3 hours
DOL 5
Discontinue PIV
BF/EBM/Similac Advance 20 Ad lib Q 2-4 hours
Heme
Physiologic Jaundice of the newborn
Mother A+, ABS negative
Infant blood type unknown
At 49 hours-Transcutaneous Bilirubin 12.4
DOL 3 - Total Bilirubin 12.8, Direct 0.2
Bili blanket started
DOL 5 - Bili blanket discontinued
DOL 6 - Transcutaneous Bilirubin 10.5
Neuro
Suspected Trisomy 21 (Intrauterine Diagnosis)
DOL 1 –FISH probe sent
Confirmed Trisomy 21 DOL 5
DOL 1- High resolution chromosomes sent
Pending results at discharge on DOL 6
Discharge
Information given on Sharing Down Syndrome AZ
Infectious Disease
No issues. Mom GBS -, ROM at delivery
Vitamin K IM injection, 1 mg x 1
within 1 hour of birth
Erythromycin ophthalmic eye
ointment to both eyes x 1 within 1
hour of birth
D10W @ 10cc/hr (80ml/kg/day)
Hepatitis B vaccine 5mcg IM x 1
Little noses Q 4 hours x 24 hours
COARCTATION OF THE AORTA
Until surgery can be performed PGE is needed.
Surgical correction has the lowest risk of restenosis then any
other treatment.
Although surgical correction reduces the morbidity and
mortality rate. These infant’s still have a decreased life
expectancy related to poor blood pressure control.
Outcome is worse when there is also a hypoplastic arch present.
If the ECHO is performed by someone whom is not experienced
in neonates and small children a CT or cardiac MRI should be
performed to officially adequately assess the aortic arch.
Surgical intervention is the preferred method of treatment
because those whom have a balloon dilation will almost always
need a repeat dilation and/or surgery.
(Shah, 2014)
(Panzer & Dewolf, 2014)
TRISOMY 21
Currently there is no cure for Down’s syndrome.
Genetic studies are focuses on finding therapies to improve
learning and potential therapies.
Early intervention programs, physical therapy, occupational
therapy, and speech therapy can improve outcomes.
At greater risk for hearing, vision, and learning deficits.
Leukemia is more common with Trisomy 21 patients then those
without.
(Heyn & Perlstein, 2014)
Mother and father were concerned immediately
after birth, questions answered and plan of care
explained. Infant skin to skin with mother prior
to transfer to NICU for further evaluation.
Father accompanied infant to NICU and further
questions answered. After recovery mother
visited infant in the NICU, asked appropriate
questions regarding suspected Coarctation and
Trisomy 21.
Discharge home with parents 6/3/14 DOL 6
Diet: EBM/ Similac Advance 20cal/oz ad lib q 2-4 hours
1st Newborn screen sent 5/29 results pending
2nd Newborn screen sent 6/1 results pending
ABR hearing screen passed on 6/2/14
Hepatitis B Vaccine given on 6/1/14
Follow up with Primary Care Physician in 2days
Follow up with Pediatric Cardiologist in 1-2 weeks
Coarctation is a constricted segment of the aorta
that comprises localized medial thickening and
can be life threatening if not diagnosed and
treated.
A prenatal diagnosis of Coarctation of the aorta
can assist the medical professionals in the
preparation for treatment.
Trisomy 21 is a genetic defect that may have
multiple associated anomalies.
Confirmation of a prenatal diagnosis is important
to assist the family in the acceptance of a child
with a chronic disorder and/or a genetic
disorder.
Although it is common for a Trisomy 21 infant to
have an associated heat defect it is not
commonly associated with coarctation of the
aorta.
Bajaj, K & Gross, S. (2011). Genetic aspects of perinatal disease and prenatal
diagnosis. In R. J. Martin, A.A. Fanaroff, & M.C. Walsh. Neonatal-Perinatal medicine:
Diseases of the fetus and infant (9th edition pp.129-145). Volume 1. St. Louis,
Missouri: Elsevier Mosby
Blackburn, S. (2007). Maternal, Fetal, & Neonatal Physiology (3rd edition). St. Louis,
Missouri: Saunders Elsevier.
Cates, L.A. (2009). The basics about acyanotic heart disease (Powerpoint
presentation).
Gholampour, D.M., Givtaj, N., Omrani, G.H., Sadeghpour, T.A., & Yaghoobi, A. (2006)
Evaluation of results of surgical correction for coarctation of aorta by suclavian flap
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diseases, and drugs (7th edition). New York: McGraw Hill Education.
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http://www.onhealth.com/down_syndrome/article.htm
Jones, K.L. (2006) Smith’s recognizable patterns of human malformation. (5th edition)
Philadelphia, Pennyslvania: Saunders Elsevier.
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2014, from, http://emedicine.medscape.com/article/150369-overview
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Retrieved May 31, 2014 from http://emedicine.medscape.com/article/895502overview