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Morning ConferenceMeghan OConnor

Monday, June 25, 2012

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7 day old girl withrespiratory distress


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History

HPI:

Increased work of breathing with feeds since birth, but nasal saline drops

and bulb suction seemed to help.

Rapidly worsened tonight.

Started breathing fast at baseline

Irritability

Difficulty with feeding: 1-2 sucks before needed to come off the breast

and breath for several seconds.

Pale and dusky in color


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History

PMHx: No hospitalizations or surgeries.

Birth Hx: NSVD at term without complications.

Meds: None

Allergies: NKDA

FamHx: No chronic disease in children.

SocHx: Lives with parents. No sick contacts.


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Physical Exam

Vitals: T 35.8 HR 192 RR 102 BP 83/56 SAT 98%

BP: RUE 83/56 LUE 79/52 RLE 59/44 LLE 58/46

GEN: Distressed infant. Irritable.

CV: Tachycardia, regular rhythm, no murmur, poor perfusion, 2+ upper

extremity pulses, difficult to palpate lower extremity pulses

RESP: Significant respiratory distress. Lungs clear to auscultation bilaterally,Tachypneic.

FEN/GI: Liver edge palpated 4cm below the right costal margin, abdomen

distended but soft, no splenomegaly


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Laboratory Evaluation

CBG: 7.35/42/49/22/-3

Lactate: 3

CBC: WBC 13.4 (27%N 53%L) HCT 44.1 PLT 311

CMP: Nl


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Imaging

ECG: RVH

CXR: Cardiomegaly with increased pulmonary

vasculature


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7do with respiratorydistress & hypothermia


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0Single Ventricle Pathology


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1Truncus Arteriosus


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2Transposition of the GreatArteries


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3Tricuspid atresiaCritical tricuspid stenosisEbsteins Anomoly


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4Tetrology of Fallot

Critical pulmonic stenosis

Pulmonic atresia

Aortic atresia

Critical aortic stenosis

Coarctation of the Aorta


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5Total Anomalous PulmonaryVenous Return


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Physiology of cyanotic CHDDecreased pulmonary blood flow

Tetralogy of Fallot, pulmonary valve atresia,

critical pulmonary valve stenosis, and tricuspid

valve abnormalities

Increased pulmonary blood flow:

Transposition, truncus arteriosus, and TAPVR

Heart Failure:

HLHS, coarctation and interruption of the aorta,

and critical valvar aortic stenosis.


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Coarctation of the Aorta


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Prevalence & Associations

6-8% of all children with congenital heart disease

Males affected 2x more than females

Increased incidence in Turner Syndrome (~10%)

Increased incidence of intracranial hemorrhage

Up to 75% have an associated bicuspid aorticvalve

Other common accompanying defects: VSD,

aortic valve stenosis, mitral valve stenosis, PDA


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Presentation:Newborn- 50% of cases

Physical: delayed or diminished femoral pulses; +/-

murmur

Heart failure/cardiogenic shock: pale, irritable,diaphoretic, dyspneic, absent femoral pulses, +/-

hepatomegaly

Older Children

Chest pain, exercise intolerance, cold extremities,

claudication with physical activity

Physical: differential pressures, femoral pulse delay,

HTNMonday, June 25, 2012

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Diagnosis:

Prenatal diagnosis

ECG

CXR

ECHO


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Managment

Critical Coarctation in

Infancy:

Prostaglandin E1

Dopamine or dobutamine

Supportive care

Surgical repair

Children:

Surgical repair


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Surgical repair

Resection with end-to-

end anastomosis

Subclavian flap

aortoplasty

Bypass graft

Balloon dilation


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Complications

Early:

Paradoxical HTN

Left recurrent nerve paralysis/phrenic nerve injury

Subclavian steal

Late:

Recoarctation (5-14%)

Aortic aneurysm (Risk increases with >age)

HTN


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Long Term

ManagementLifelong follow-up with cardiology

Screening for HTN

Imaging of the site

Exercise testing in competitive athletes

Head imaging


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References

Agarwala BN, Bacha E, Cao Q, Hijazi Z. Clinical manifestations and diagnosis of coarctation of the

aorta. Uptodate. Last updated: Dec 21, 2011.

Korbmacher B, Krogmann ON, Rammos S, et al. Repair of critical aortic coarcation in neonatal age. J

Cardiovasc Surg (Torino) 2002; 43:1.

Lu CW, Wang JK, Chang Cl, et al. Noninvasive diagnosis of aortic coarctation in neonates iwth patent

ductus arteriosus. J Pediatrc2006; 148:217.

Marino BS, Bird GL, Wernovsky G. diagnosis and mangement of the newborn with suspected

congenital heart disease. Clin Perinatol 2001, 28:91.

Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Manaagmenet fofAdults with Congenital Heart Disease: a report of the American Heart Association Task Force on

Practice Guidelines. Ciculation 2008, 118e714.

Heart Institute Encyclopedia-Cincinnati Childrens Hospital. http://www.cincinnatichildrens.org/

patients/child/encyclopedia/default/.
http://www.cincinnatichildrens.org/patients/child/encyclopedia/default/http://www.cincinnatichildrens.org/patients/child/encyclopedia/default/http://www.cincinnatichildrens.org/patients/child/encyclopedia/default/http://www.cincinnatichildrens.org/patients/child/encyclopedia/default/http://www.cincinnatichildrens.org/patients/child/encyclopedia/default/

coarctation 06.26.2012

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