Cardiology in the DNCC

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Cardiology in the DNCC

• Julie Martchenke, RN, MS, PNP

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Normal Transition from Fetal Circulation
Ductus closes, Pulm. Vasc. Resistance drops
Ductal flow reverses, Branch PAs open up
Ductal Flow PA to descending Aorta,
branch PAs tiny

• 2-3 months of age

• Fetal Circulation

• 8 Hours old

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Congenital Heart Disease Types

• Lesions with Increased Pulmonary Blood Flow ? CHF
  or ?PVR
• Lesions with Decreased Pulmonary Blood Flow
• Lesions with Inadequate Systemic Flow

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I. Increased Pulmonary Blood Flow Lesions (L ?R
Shunts)

• PDA
• VSD
• ASD
• AV Canal Defect
• Some Double Outlet Right Ventricle
• Some Truncus Arteriosus

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Patent Ductus Arteriosus

• Hard on little premies, if can hold on, often
  closes near term.
• Give indocin x3, 2 courses, no success surgical
  ligation
• Big DA, big failure, can be disastrous
• Big endocarditis risk

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Pulmonary Overflow leading to Congestive Heart
Failure.

• Tachypnea, retractions,
• poor feeding and weight gain,
• hepatomegaly,
• diaphoresis,
• hyperactive precordium, ?perfusion

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CHF Treatment

• Formula Fortification
• NG feeds, feeding techniques
• Medications
• Lasix
• Aldactone
• Digoxin
• Captopril/ Enalapril

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Increased Pulmonary Vascular Resistance
Pulmonary Artery Diameters
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Eisenmengers

• Occurs with any Large Left to Right defect that
  is not corrected or is uncorrectable.
• Progressive damage to pulmonary vasculature
  lining becomes more muscular increasing PVR,
  PHTN, cyanosis--death

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VSD Case Scenario 1

• DG is a 4 week-old 34wk premie transferred to
  DNCC from outside facility with a VSD, his po
  intake has fallen off in past 2 days. He has
  been taking 11 ounces a day of 20KCal formula.
  He is on no medications.
• What would you assess on first exam?

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Case Scenario continued

• RR 80, no retractions, sweating after
  bottle-feed, lungs clear, HR 140, Liver 4-5 cm ?
  costal margin. Takes 40 minutes to take 2.5 oz.
  No weight gain past week. Good distal perfusion.
  O2 sat 93.
• What is your assessment?
• Would you give Oxygen?
• What other interventions are likely?

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Case Progression

• Baby is in Congestive Heart Failure. Oxygen is
  best avoidedwill worsen failure
• Interventions
• Calorie fortification, first 24Kcal/oz, then 27
  Kcal/0z,
• NG placed
• Digoxin, Lasix, Aldactone started
• What Teaching will you need to do?

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VSD Case Scenario 2

• Baby RB was born at 30 weeks gestation with
  moderately large VSD, and Downs syndrome. He
  was eventually able to grow and was discharged
  from Nsy after 8 weeks. He goes home on Lasix,
  Digoxin and Aldactone
• What symptoms would you instruct the family to
  look for?

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Case Scenario continued...

• Teach family signs and symptoms of Congestive
  Heart Failure, and side effects of medicines.
• Surgery is planned for 3-4 months corrected age.
  However, at 2 months corrected age mom notes the
  baby turns somewhat blue with feeds and crying.
• What might be happening?
• What further assessment would you do when the
  child is readmitted?

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Case Scenario

• Baby has possibly developed increased pulmonary
  vascular resistance (or it never dropped
  completely from Newborn period). Now there might
  be right to left shunting with exertion.
• Sats drop to mid-80s with feeds and crys, echo
  shows right to left shunt with cry.
• What will be probable plan?

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Other Increased Pulmonary Blood Flow Lesions
Atrial Septal Defect

• Often doesnt show up for 1 yr.
• Pulmonary Vascular DZ risk 10 if not corrected
• Usually correct age 3-4

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Atrioventricular Canal (or Endocardial Cushion
Defect)

• Often have Downs Syndrome
• Partial or Complete Types
• Usually have large Left to Right shunts

1ASD
Common AV Valve
VSD
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AV Canal Surgical Repair

• Atrial and Ventricular defects are patched and
  Mitral and Tricuspid valves made out of common
  valve
• AV canal repairs are associated with possible
  pulmonary vasospasm and AV valve problems in
  addition to usual open-heart surgery
  complications.

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II. Cardiac Lesions With Inadequate Pulmonary
Blood Flow

• Tetralogy of Fallot
• Pulmonary Atresia with VSD (Severe Tetrology)
• Critical Pulmonic Stenosis
• Some Truncus Arteriosus defects
• D-Transposition of Great Arteries
• Severe Ebsteins Anomaly

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Tetrology of Fallot

• Usually not cyanotic at birth, becomes cyanotic
  over time espec with crying.
• Usually repaired surgically 3-6 months of age

Overriding Aorta
VSD
PS
RVH
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Surgical Repair of Tetrology

• VSD patch and Transular Patch to open
• Post surgical risks for arrhythmias and heart
  blocks in addition to usual open heart surgery
  complications

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Pulmonary Atresia with VSD

• In Pulmonary Atresia with VSD, the main pulmonary
  artery never forms or is tiny. When the ductus
  closes there is no pulmonary blood flow and the
  baby gets cyanotic

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Pulmonary Atresia with VSD repair

• May need aorto-pulmonary shunts first to grow
  branch PAs
• Risk of arrhythmias, blocks, low cardiac output
  in addition to nl bypass risks

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Critical Pulmonic Stenosis

• In critical PS,the pulmonic valve is very narrow
  and stenotic. The RV is unable to pump adequate
  blood flow to the pulmonary artery, therefore
  desaturated blood backs up to the RA and crosses
  the Patent Foramen Ovale to the LA. The child is
  cyanotic from birth and this worsens when the
  Ductus arteriousus closes.

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Truncus Arteriosus with Pulmonic Stenosis

• In Truncus Arteriosus the fetal truncal artery
  never completed dividing into the aorta and
  pulmonic artery.
• Blood from the RV and LV both exit through the
  truncus and, in some cases, some blood will pass
  into a pulmonic artery takeoff partway up the
  ascending truncus.
• This pulmonic artery takeoff can be wide open and
  flood the lungs or it can be narrow and stenotic
  causing inadequate pulmonary blood flow and
  cyanosis, especially when the Ductus closes.

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D-Transposition of Great Arteries

• Great arteries reversed, not compatible with life
• May have VSD, PFO, DA for mixing

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Arterial Switch Repair

• Great arteries switched
• Coronaries must be carefully moved
• Post-op watch for bleeding (many sutures),
  ischemia of coronaries

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Open heart Surgical cases On CardioPulmonary
Bypass
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Cardiopulmonary Bypass (CPB)

• Cardiopulmonary Bypass is used to support
  respiratory and circulatory function of child
  while the surgeon is operating within the heart.
• Cannulae are put in IVC and SVC, where they drain
  blood into the Bypass circuit. There the blood
  is oxygenated and pumped back via another cannula
  to the Aorta. By this process blood bypasses the
  heart and lungs.

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CPB Circuit
IVC and SVC Cannulae
Aortic Cannula
oxygenator
pump
Rtn to Body
Heater/Cooler
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Complications of CPB All Patients on Bypass Have
These Risks

• Air and Particulate emboli can get into blood
• There is often a whole Body Immune Response to
  the CPB tubing leading to leaky capillaries and
  EDEMA.
• A Coagulation Cascade is initiated leading to
  clotting factor consumption and bleeding.
• RBCs are traumatized by pump system --leading to
  hemoglobinuria and anemia

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More CPB Effects

• Arrhythmias are possible due to trauma of
  instruments, area of surgery, or stun from heart
  arrest
• Poor cardiac function will occur to some degree
  (and worse with longer pump runs) due to heart
  arrest

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Cardiopulmonary Bypass Edema

• Example A 3.5 kg infant (280 ml circ. Blood
  volume) will gain 600 ml.
• Edema worse with longer bypass time and younger
  patient. This will be hopefully reduced as we use
  Ultrafiltration and or Solumedrol pre-op in
  children under 6 months.
• Edema increases over 1st 24-48.Then diuresis
  usually begins. U/O may fall below 1cc/kg/hr
  during cap. leak phase.

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Electrolyte Imbalances Post Operatively

• Increased Na secondary to NS in prime
• Decreased K secondary to water loss
• Increased K secondary to renal failure
• Decreased Ca in young infants due to poor
  kidney regulation and citrate in blood received
• Decreased glucose due to stress and NPO in young
  infants
• Increased glucose if glucose in prime

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Ebsteins Anomaly

• Tricuspid valve displaced low in RV
• Very regurgitant
• Shunting R?L thru Foramen Ovale
• Sometimes inadequate pulm flow without ductus

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Case Scenario 3

• 2 wk old with severe Ebsteins Anomaly noted to
  have increased HR to 210 at beginning of your
  shift.
• What further assessment would you make?

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Case continued

• Assessment Baby warm, well perfused initially,
  pulses 3 femoral, brachial, BP 59/37
• Meds on Digoxin 5 mcg bid, flecainide 5mg bid,
  PGE 0.05mcg/kg/min
• EKG

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Case 3 continued

• Diagnosis Reentrant Supraventricular Tachycardia
  due to accessory pathway (common problem with
  Ebsteins anomaly)
• What interventions would you anticipate?

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Interventions for SVT

• Emergent
• Adenosine 0.1mg/kg (may repeat 2-3 times)
• Esophageal pacing or transthoracic pacing (rate
  250) to overdrive reentrant circuit rhythmn
• Chronic
• Various antiarrhythmics, dig, inderol,
  flecainide, amiodorone,

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Case Progression

• After 3 hours with multiple doses of adenosine,
  baby finally converted by esophageal pacing by
  pediatric EP cardiologist.
• BP now 68/37, HR 150
• Ekg
• Chronically child eventually discharged on
  Flecainide
• (incompatible with milk)
• Expensive (prior auth for OHP/insurance)
• Toxic ( proarrhythmic, cns, liver enzymes, )

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III. Lesions with Inadequate Systemic Blood
Flow...

• Critical Coarctation
• Hypoplastic left ventricle
• Interrupted aortic arch
• Critical aortic Stenosis

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Critical Coarctation

• Tight narrowing in the aorta right above the
  ductus arteriosus which constricts blood flow to
  the descending aorta.
• When the ductus closes these babies have very
  compromised flow to the abdomen and lower
  extremities.

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Case Scenario 4

• DW is a 2 kg, 2 day old (28 wk gest) transferred
  to DNCC from outside facility for critical
  coarctation
• What exam findings and medications should you
  expect?

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Case Scenario Progression

• Watch for shock and acidosis, poor lower
  extremity perfusion.
• Should be on PGE to reopen or maintain ductus to
  get some flow to lower extremities and probably
  loosen the coarct.
• Prepare for surgery.

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Coarctation Repair

• Left-sided thoracotomy incision to access the
  aorta.
• Coarctation is cut away and the two ends are sewn
  together (anastamosed). Sometimes a gortex patch
  used.
• Cardiopulmonary bypass not necessary but may be
  if anatomy is complex.
• Key issue is cross-clamp time.

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Surgery

• What information should the DNCC staff obtain
  about the child before she/he returns from
  surgery?

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Info to Gather Prior to Arrival

• Preoperative health status
• Intraoperative events
• Know if pump run (rare) and time, cross-clamp
  time
• Any complications with surgery (anti-arrhythymias,
  inotropes, packed cells needed?)
• Particular post-surgical complications associated
  with specific surgery

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Possible Complications Post-op With Coarctation
Repairs

• Bleeding at suture lines.
• Damage to the laryngeal nerve that causes
  transient vocal cord paralysis. This usually
  resolves in 6 months.
• Damage to the left phrenic nerve leads to
  elevation of the left hemidiaphragm.
• Mesenteric artery and or renal artery ischemia
  from cross-clamping causes abdominal pain,
  distension and tenderness. Ischemia may also
  cause ATN.
• Ischemia to spinal cord blood supply during cross
  clamping may lead to paraplegia (rare).
•         

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Post-op Coarct complications continued

• CVAs occasionally occur also during surgical
  cross-clamping.
• Post-coarctation syndrome occurs in some
  patients, with postoperative hypertension that
  tends to resolve in 24 hours.
• Managed with nitroprusside drips and propranolol.
  
• Hypertension can again occur 48-72 hours after
  surgery. Second stage hypertension often has
  higher diastolic pressures which can be serious.

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Coarctation Case Scenario Continued

• This infant had prolonged repair d/t difficult
  anatomy. Cross-clamp time total 3 hrs and 4
  minutes with 61 minutes off between 3 clamps.
• First night post-op u/o 10 cc, I/O 180/36 for 24
  hrs. Urine rose colored.
• What would you do if you noted this?

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Case scenario

• Notify Cardiology
• Draw Labs Na 131, K 6.1, cl 100, BUN/Creatinine
  12/1.8
• VSS
• What is your Assessment?

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Acute Renal Failure D/t Acute Tubular Necrosis
D/t Prolonged Cross-clamp Time

• Plan
• No K
• Restrict fluid intake
• Lasix 2mg/kg q 12
• Labs q 4-6 hrs
• Wait, hope it gets better, if not dialysis (if
  oliguric)
• Look for concomitant ischemia

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Case 4 Progression

• Max BUN/Cr 50/3.1 then slowly came down, In NICU
  3 wks, on outpatient visit 6 weeks after surgery
  labs finally normal.
• No hepatic or enteric complications developed.

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Hypoplastic Left Heart Syndrome

• In Hypoplastic Left Heart the LV is
  underdeveloped. Often the LA, mitral valve,
  aortic valve, and aortic arch are also small.
• The newborn is dependant on the Ductus to
  maintain perfusion, when it closes the baby will
  become shocky and acidotic

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Norwood Procedure (Stage I HLHS repair-newborn)
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Glenn Anastamosis (Stage II 6mos of age)
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Fontan Procedure(Stage III 2-4 years of age)
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Post-op Norwood Repair

• At OHSU, babies stay on ECMO or NOMO for 2-7
  days post-op until cardiac function has
  stabilized. Then weaned off and transferred to
  DNCC.
• Systemic output/ pulmonary output balance should
  be reached by this point, but can be affected by
  too much oxygen or
• too little cardiac output.

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Being on ECMO

• Ventricular assist (partial).
• At first 200/cc/kg (80of blood vol).
• Then wean down to 150-100 before trial off (40
  blood volume).
• Respiratory assist.
• Ventilator turned down to very minimal settings
  (but still moving), oxygenation and CO2 removal
  happening in circuit.
• Goal is minimal oxygen (but totally dependant on
  baby).
• When trialing off ventricle assist, ventilator
  settings go up to maximal settings.

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ECMO

• Remember they have an open chest and big
  catheters in heart (8 and 10 fr catheters)
• Big infection risk
• Totally changes pressure dynamics

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Post-ECMO Norwoods Continued

• Sats should be 70, ideally mid-80s. If not look
  for
• Pulmonary problem,
• Poor ventricular function,
• Inadequate cardiac output d/t inadequate volume,
• Inadequate pulmonary blood flow d/t shunt
  obstruction (clot, kink) or high pulmonary
  pressures.
• Main challenge for these children is eating.

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Case scenario 5

• You receive a term baby post-op day 6 from PICU
  for a Norwood procedure.
• What questions do you need to ask of transferring
  PICU staff?

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Post-Norwood Questions

• Pre-surgical health state
• Complications with surgery
• How many days of ECMO
• Baseline O2 saturation off ECMO
• Current status, intubated, chest tubes etc.

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Case 5 continued

• Child did well post-op, on ECMO 2 days only, sats
  now low 80s, intubated, near dry weight, chest
  tubes out.
• During your shift child is extubated and does
  well, continues same meds.

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Case 5 Continued

• During your next shift with the baby you note
  sats are now sometimes high 60s to low 70s. RR,
  HR continue same (high normal), child is starting
  tube feeds.
• What might be happening?
• What would you assess?
• What interventions would you do?

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Scenario continued..

• Sats could fall d/t pulmonary causes or cardiac
  causes
• Poor ventricular function
• Inadequate cardiac output d/t inadequate volume
• Inadequate pulmonary blood flow d/t shunt
  obstruction (clot, kink) or high pulmonary
  pressures
• Assess for both pulmonary and Cardiac causes

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Interventions

• 100 O2 not the answer. Oxygen causes pulmonary
  vasodilation which could steal from systemic
  circulation. However, profound hypoxemia not
  acceptable either. Often more volume or better
  cardiac function helps with some O2.

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Case 5

• Child given fluid bolus 20cc/kg- O2 sats improved
  to high 70s on ¼ ltr nc.
• Child was volume depleted ? ?Oxygen extraction at
  capillaries ? ? mixed venous sat ? ? mixed
  arterial sats.

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Interruption of the Aortic Arch

• Severe form of coarctation with no continuation
  of the aortic arch to the descending aorta.
• If the ductus closes the child has no flow to the
  lower extremities and may become severely ill and
  die.

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Critical Aortic Stenosis

• Aortic valve is often small as well as stenotic
  and narrow.
• LV is small and the ascending and transverse
  aorta can be very small.
• Blood flow to the lower extremities from the RV
  via the Ductus Arteriosus. The child will get
  very acidotic and have poor perfusion when the
  Ductus closes.

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Aortic Valve Repair

• For stenosis balloon valvotomy
• Still have high risk of sudden death
• Watch for bleeding and arrhythmias post op
• Ideally should see improved perfusion and energy
  in baby

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Congenital Complete Heart Block (case 6)

• YD is a 26 y.o. woman 26 weeks pregnant. Fetal
  HR noted to be 70. Fetal echo done, shows atria
  and Ventricles beating separately.
• What causes this?
• What would you assess for when this baby is born?

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• EKG indicates 3rd degree heart block
• Usually d/t Maternal antibodies to connective
  tissue dz, anti rho,la OR Surgery in area of AV
  Node
• Assess for CHF
• Tx Pacemaker if necessary

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The End