*

Aaron McCoy, MD, PGY3

* 9 day old male brought to the ED with concerns about

decreased and difficult oral intake and grunting respirations. for grunting and oxygen need but this improved quickly and he was discharged home on 1/16L 02.

* Was delivered at an OSH and was admitted to the NICU there * Per parents, had an ECHO in the NICU for a murmur and
developed difficulty breathing and difficulty feeding.

respiratory difficulty showing a PFO and bicuspid aortic valve.

* He was fine for a few days but the day prior to presentation * No fevers. Appropriate number of wet diapers and stooling.

* PMH/BH: Term, uncomplicated pregnancy and

delivery. Benign prenatal US.

* FH: PGM with hole in her heart, multiple

paternal family members with asthma.

* SH: Lives in UT with parents and 20 m.o. sister. * Medications: None * Allergies: NKDA

* Vitals: T 36.6 RR 40 HR 180 BP not obtained Sats 96% on 1/16L * General: He is ill appearing, pale, diaphoretic, moderate distress * Extremities: Cool, delayed cap refill at 8 seconds * HEENT: Normocephalic, atraumatic, AF somewhat sunken, TMs

* * * *
* *

clear, clear conjunctiva, no rhinorrhea, lips are dusky, no LAD CV: Tachycardic, S1, S2, no murmur Resp: Mild increased WOB, clear to auscultation, symmetric Abd: Soft, non-tender, non-distended, hepatomegally palpable Neuro: No apparent focal deficit in sensation, motor, or cranial nerves, no pain with neck flexion Skin: No rash or lesion GU: Normal exam, tanner stage appropriate for exam

* VBG 7.20/61/33/24.0/5 * Lactate 9.5 * CMP Na 134, K 3.0, Glucose 273, Tbili 6.7, AST
64 (after fluid resuscitation)

* CBC: WBC 10.8, HCT 38.6, PLTS 331

* CXR: Mild cardiomegaly and pattern of central

perihilar opacity suggesting possibility of early congestive heart failure.

* ECHO: Juxtaductal coarctation of the aorta,

small PDA with L to R shunting, bicuspid aortic valve, dilated RV with severely diminished function, normal LV and function, PFO with L to R shunting

* Typically located at the insertion of the ductus

arteriosus just distal to L subclavian artery, almost always congenital * Results in LV pressure overload * Prevalence in 4/10,000, about 5% of CHD * Associated with other cardiac lesion (93%) * Does not cause intrauterine compromise because majority of blood goes through DA and bypasses coarctation

* As DA and FO close the blood must cross the

heart failure

isthmus resulting in increased systolic pressure

* Range of symptoms from mild systolic HTN to * Compensatory mechanisms include LVH and
collaterals (intercostal, internal mammary, scapular). Heart failure develops when systolic pressure is too severe to allow time for compensation

* Can be asymptomatic with PDA or if coarct is not

severe. * Clinical diagnosis is made by absent or delayed femoral pulse (brachio-femoral delay) * Murmur may be present but may not because it is usually from other cardiac defects * Other clinical signs are pallor, irritability, diaphoresis, dyspnea, hepatomegaly * CXR: notching of posterior third of ribs 3-8 (erosion of collaterals into rib), 3 sign narrowing with pre and post dilation * Confirmed with ECHO

* Diagnosis often delayed because most are

asymptomatic coarctations

* May present with complaints of chest pain,

cold extremities, and claudication with exertion

* 4 point blood pressures * Mild cases may present in adults as HTN

- Started on PGE to keep ductus open and transferred

to the PICU. - Intubated shortly after arrival to PICU for respiratory distress - Surgical correction the next day - Seen in Cards clinic 2 mo ago (6mo age), growing and developing well, no concerns, most recent ECHO shows normal ventricles and normal function - Seen in genetics clinic where it was recommended all 1st degree relatives get ECHO