Coarctation of the Aorta - PowerPoint Presentation

Slide1

Coarctation of the AortaTrisomy 21Clinical Case Presentation

Presented by: Lynette Barnhart RNC, BSN, SNNPJune 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

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ObjectivesMaternal HistoryMaternal and Fetal Risks and Complications

Delivery and StabilizationAdmission exam and diagnosticsPrimary admission diagnoses Etiology and Pathophysiology of Primary Admission Diagnoses Initial Plan of CareHospital

Course by Symptoms

Medications

Pertinent

Theories and Evidence Based Practice

Family

Interactions

Discharge

Plan and Follow Up

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Maternal History37 yo

, married HispanicG6 now P51 SAB1994-39 weeks1997-40 weeks2004-39 weeks2009-39 weeksEDD 6/10/14

A+, GBS-, RPR NR, Rubella Immune, HIV NR, HB-

Unremarkable Medical history

Denies alcohol, tobacco, and illicit drug use

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Maternal & Fetal Risks & ComplicationsAdvanced maternal age

37 year oldU/S 12/16/14Nuchal thickness of 3.1Low AFP 12/31/14+ Trisomy 21U/S 2/28/14Suspected Coarctation of the aorta

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Delivery & StabilizationScheduled induction at 39 weeks

Due to suspected Coarctation of the aortaSpontaneous labor38 1/7 weeks GASROM 2 ½ hours prior to deliveryClear fluidVertex presentationNSVDNo 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 pre-ductal 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.

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Admission Assessment Gestational age

38 1/7 weeksMeasurementsBirth weight: 3078gms (10th-50th %tile)

Length: 48.5 cm (10

th

-50

th

%tile)

OFC: 32 cm (10

th

%tile)

Vital signs

Temperature 36.9 C

Heart rate 120 bpmRespiratory rate 40

br

/min

Pre-ductal SpO2 94%Post-ductal SpO2 89%

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Admission Assessment Physical Exam

General: Habitus of Trisomy 21, No acute distress, active with exam, sucking on pacifierEye: 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.

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Admission DiagnosticsEchocardiogram5/28/2014-

1 hour of lifeFISH chromosomes5/29/2014- results pendingHigh resolution chromosomes5/29/2014- results pending

Bedside

chemstrip

51 @ 1426 (admit)

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Primary Admission Diagnoses

38.1 week AGA male infant, 3078gmsSuspected Coarctation of the aortaSuspected Trisomy 21

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Etiology and Pathophysiology of Coarctation of the Aorta

Narrowing of the aortaConstricted segment of the aorta that comprises localized medial thickening.5-8% of all congenital heart defectsMay also occur with bicuspid aortic valve and VSD

(Cates

,

2009)

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Coarctation of the Aorta

Increased afterload on the left ventricleIncreased wall stress resulting in ventricular hypertrophyAs 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)

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Coarctation of the Aorta

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Coarctation of the Aorta

Clinical findingsSystolic ejection murmur that radiates to the apex and is often loudest in the backXray finidngsGeneralized 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

Cyanotic lower extremities

(

Gomella

, 2013)

(

Syamasundar

, 2012)

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Coarctation of the AortaMedical Treatment

Prostaglandin E 0.05-0.15 mcg/kg/minVentilatory assistanceTo support increased work of breathingInotropic drugs

Dopamine,

D

obutamine

,

E

pinepherine

Diuretics

Used for infants with CHF

Foley Catheter

Strict monitoring of urine output and renal perfusion

UAC

ABGs

(Cates, 2009)

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Coarctation of the Aorta

Surgical TreatmentGoal is to bypass the narrowed section of the aortaLeft subclavian aortoplastyResection and end-to-end anastomosisAngioplasty with Dacron patchStenting

Balloon angioplasty

(

Gholampour

, et al, 2004).

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Trisomy 21Down Syndrome

Presence of an extra 21st chromosomeMost common of all multiple congenital anomaly (MCA) syndromesOccurs in ~ 1 in 650 birthsNeonatal 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)

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Trisomy 211

st trimester screeningUltrasound between 10 4/7 and 13 4/7 weeks gestationMeasurement of the nuchal translucency (increased in Trisomy 21)Detects 70% of Trisomy 212nd 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% detectionDecreased AFP, increased

hCG

, decreased PAPP-A, and increased

Inhibin

A

(Bajaj & Gross, 2011)

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Trisomy 21Physical 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

anomalies.

(

Gomella

, 2013

)

(Jones, 2006)

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Trisomy 21TreatmentThere is no cure for Down’s syndrome

Supportive careEarly intervention is essential to assist in the progression of developmental milestones.(Heyn &

Perlstein

, 2014)

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Initial Plan of CareAdmit to NICU, place on continuous cardio/

resp monitoringDaily weight, OFC and Length Q SundayErythromycin ointment to eyes bilaterally within 1 hour of birthVitamin K 1mg IM x1 within 1 hour of birthPlace 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

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Initial Plan of CareChemstick

on admission and then per policyObtain Metabolic Screen at 24-48 hoursObtain Metabolic screen #2 at 5-10 days of ageMay breast feed ad lib on demandSocial service consult

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Hospital Course by Systems

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

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Hospital Course by Systems

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

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Hospital Course by Systems

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.

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Hospital Course by Systems

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

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Hospital Course by Systems

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

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Hospital Course by Systems

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

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Medications

Vitamin K IM injection, 1 mg x 1 within 1 hour of birthErythromycin ophthalmic eye ointment to both eyes x 1 within 1 hour of birthD10W @ 10cc/hr

(80ml/kg/day)

Hepatitis B vaccine 5mcg IM x

1

Little noses Q 4 hours x 24 hours

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Pertinent Theories and EBP

COARCTATION OF THE AORTAUntil 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)

Slide30

Pertinent Theories and EBP

TRISOMY 21Currently 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)

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Family InteractionsMother 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.

Slide32

Discharge Plan and Follow-upDischarge home with parents 6/3/14 DOL 6

Diet: EBM/ Similac Advance 20cal/oz ad lib q 2-4 hours1st Newborn screen sent 5/29 results pending2nd

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

Slide33

ConclusionCoarctation 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.

Slide34

References

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 MosbyBlackburn

, S. (2007). Maternal, Fetal, & Neonatal Physiology (3

rd

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

aortoplasty

(SCFA) and other methods in

Rajaee

Heart Center. Retrieved May 31, 2014, from, http://www.plan.sid.ir/en/ViewPaper.asp?ID=40885&vRadif=17&vWriter=GHOLAMPOUR%20DEHAKI%20M.,GIVTAJ%20NADER,OMRANI%20GH.,SADEGHPOUR%20TABAEI%20A.,YAGHOOBI%20A.&vJournal=RAZI+JOURNAL+OF+MEDICAL+SCIENCES+%

28JOURNAL+OF+IRAN+UNIVERSITY+OF+MEDICAL+SCIENCES%29&vDate=Winter%202006&vVolume=12&vNo=49&vStart=0&vEnd=0

Gomella

, T.L. (2013) Neonatology: Management, procedures, on-call problems, diseases, and drugs (7

th

edition). New York: McGraw Hill Education

.

Slide35

ReferencesHeyn, S.N. &

Perlstein, D (2014). Down Syndrom. Retrieved June 6, 2014 , from http://www.onhealth.com/down_syndrome/article.htmJones, K.L. (2006) Smith’s recognizable patterns of human malformation. (5th edition)

Philadelphia,

Pennyslvania

:

Saunders Elsevier.

Panzar

, J. &

Dewolf

, D. (2014) Hypertension after

Coarctation

repair.

Retrieved June 6, 2014, from, http://cardiologyacademicpress.com/soap/pdf/delme_427_535c2c62225e71.97600142.pdf

Shah, S. N. (2014). Aortic

coarctation

treatment & management. Retrieved June 6, 2014, from, http://emedicine.medscape.com/article/150369-overview

Syamasundar

Rao, P. (2012, February 1)

Coarctation

of the aorta.

Medscape.

Retrieved May 31, 2014 from http://emedicine.medscape.com/article/895502-overview