1 / 70

Hypoplastic Left Heart Syndrome (and the single ventricle repair)

Hypoplastic Left Heart Syndrome (and the single ventricle repair). Henaro Sabino, MD Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta; Emory University. CHEST PAIN, SYNCOPE. “I’M COMIN’ HOME!”. GOALS. Appreciation of history of Hypoplastic Left Heart Syndrome.

judd
Download Presentation

Hypoplastic Left Heart Syndrome (and the single ventricle repair)

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Hypoplastic Left Heart Syndrome (and the single ventricle repair) Henaro Sabino, MD Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta; Emory University

  2. CHEST PAIN, SYNCOPE • “I’M COMIN’ HOME!”

  3. GOALS • Appreciation of history of Hypoplastic Left Heart Syndrome. • Basic anatomy & physiology. • (DE-mystify Hypoplastic Left Heart Syndrome.) • Understand the LOGIC behind the management of HLHS (& single ventricle lesions in general). • KEEP YOU ALL AWAKE FOR THE NEXT 1.25 HOURS.

  4. Hypoplastic Left Heart Syndrome • Spectrum of underdevelopment of the left ventricular cavity. • Have underdeveloped aortic & mitral valves (stenosis or atresia). • Left ventricle is unable to support systemic circulation (and, therefore, right ventricle is used as the single ventricle).

  5. History of HLHS • First described by Maurice Lev in 1952. • Term used by Noonan & Nadas in 1958. • Options offered: • Comfort care • Staged palliative repair, i.e. “Norwood procedure” • First successful 3-stage completion in 1983 (after multiple surgeries from 1979). • Cardiac transplant • First successful cardiac transplant: Bailey, Nov. 1985

  6. 1980s

  7. Xenotransplantation, “Baby Fae” • Dr. Leonard Bailey, Loma Linda University Medical Center, November 1984. • http://www.babyfae.com

  8. Anatomy

  9. HLHS Epidemiology • Low incidence of 1.6 to 3.6 per 10,000 live births, BUT causes 23% or cardiac deaths during 1st week of life and 15% during the 1st month of life. • Makes up about 2-4% of congenital heart disease. • More commonly males (55% to 67%). • With ONE affected child, recurrence risk is about 0.5% to 2%. • 12% prevalence of left-sided obstructive lesions in 1st degree relatives. • 15-30% incidence of genetic syndromes and extracardiac anomalies in patients w/HLHS. • Genetic markers: dHAND, HRT1, HRT2, NOTCH. • Moss & Adams, 2008.

  10. PATHOPHYSIOLOGY • Cardiac development: “Flow begets growth.” • Altered flow through the left side of the heart: • Reduced/altered flow across the foramen ovale. • Aortic or mitral obstruction.

  11. Typical Clinical Presentation • Known Congenital Heart Defect • Prenatal Diagnosis • Unknown Congenital Heart Defect • Normal pregnancy, labor and delivery • Clinically doing okay until the PDA closes ** • Cyanosis that does not improve with oxygen • Many have no other obvious anomalies

  12. DUCTAL-DEPENDENT LESION • PDA needed to: • Provide systemic perfusion • HLHS ** • Critical aortic stenosis • Provide pulmonary blood flow • Tricuspid atresia • Pulmonary atresia • Provide mixing of oxygenated & deoxygenated blood • Transposition of the Great Vessels

  13. Hyperoxitest • ABG is measured on room air. • Patient is placed on 100% oxygen (intubated) for 10-15 minutes, then ABG is repeated. • If problem is respiratory (i.e. hypoventilation), then PaO2 improves (usually above 200mmHg). • If problem is cardiac (i.e. right-to-left intracardiac shunt), there is little improvement of PaO2. • Primary pulmonary hypertension may also result in little improvement of PaO2. • (Oxygen may hasten closure of PDA!)

  14. Positive Hyperoxitest • Seriously consider initiation of prostaglandin (PGE) at a low dose (0.03 mcg/kg/min) until diagnosis is confirmed.

  15. Initial Assessment • ALWAYS • A - Airway • B - breathing • C – circulation CXR and ECG usually not very helpful in Dx.

  16. Physical Findings • Comfortable or in distress? • Cyanosis w/out respiratory distress is cardiac until proven otherwise • Active or lethargic? • Cyanosis? • Degree - saturation usually <85% to be seen • Anemia makes cyanosis difficult to notice • Pallor • Vasoconstriction from circulatory shock • Perfusion and Peripheral pulses • End organs (i.e. watch UOP)

  17. Respiratory Status • Tachypnea but with minimal distress…cardiac until proven otherwise.

  18. Respiratory Status • Respiratory distress • Inability of the respiratory system to compensate for the metabolic acidosis • Concurrent respiratory disease • Unrelenting metabolic acidosis - decreased cardiac function • Exhaustion

  19. AssistedVentilation • Intubate if: • Impending respiratory failure • Potentially not necessary to intubate just for PGE therapy if ground transport • Intubate for air transport in PGE dependent babies

  20. Assisted Ventilation • Ventilation strategy • Volume ventilation if possible to maintain consistent minute ventilation in the face of changing lung compliance • Bigger tidal volumes compared to premature newborns (10 cc/kg); lower rates • No need to “over-ventilate” • “40/40/40 club”

  21. Arterial Blood Gases • In congenital heart disease typically: • Compensated or partially compensated metabolic acidosis • Arterial PO2 usually low <50 with cyanotic heart disease…but not always • If PCO2 is rising, think respiratory failure - be ready to intubate!

  22. Blood Gases Arterial Capillary Venous PH accurate accurate lower PO2 accurate invariable lower PCO2 accurate accurate higher HCO3(calculated) accurate accurate accurate

  23. Oxygen • Oxygen is a drug - use it with respect • Oxygen is a pulmonary vasodilator • May worsen pulmonary congestion • Oxygen is a stimulus for the PDA to close • May worsen ductal dependent lesions by speeding up closure of the PDA • Oxygen is not bad

  24. Saturation Monitoring • Oxygen saturation reflects tissue oxygenation and usually does not correlate with PO2. • With pulmonary hypertension will see differential cyanosis - shunts right to left across the PDA. • The number is not as important as the patient.

  25. Prostaglandin Infusion • Purpose is to open the PDA if a ductal dependent lesion is suspected • Can be initiated before a definitive diagnosis is established • Need a secure IV (PIV, PIC, or UVC-central or in the liver) • Start at low dose 0.03 mcg/kg/min

  26. Prostaglandins continued • Side effects - • Apnea - be prepared to intubate • Fever • Hypotension - have volume and inotropes available • Flushing

  27. Access • Umbilical is preferred in a newborn • UVC – even if in suboptimal position • UAC • PIC line • PIV • AVOID groin line if possible

  28. Fluid Resuscitation • Needed if poorly perfused • 5% albumin bolus (5-10 cc/kg) • Watch for and treat hypoglycemia - stress causes epinephrine release which increases utilization of glucose. • PRBC to treat anemia - optimize oxygen carrying capacity.

  29. Hypotension • Check ionized calcium • Treat with 50-100mg/kg calcium gluconate or 10 mg/kg calcium chloride via central access • Dopamine 3mcg/kg/min increase as needed (no higher than 10 mcg/kg/min)

  30. Metabolic Acidosis • Treat metabolic acidosis aggressively (base deficit < -3) • 1 meq/kg Na bicarbonate • Repeat blood gas

  31. Renal function Urine output BUN/Cr Renal ultrasound Head ultrasound Liver function tests Coagulopathy Thrombocytopenia R/O sepsis Genetics Other Systems

  32. Fetal Diagnosis

  33. Fetal Studies • Hornberger, 1995: 21 fetuses with prenatal echos that show left-sided obstruction (small mitral valve & ascending aorta) developed HLHS. • Critical aortic stenosis  decreased blood flow through left heart  LV dilation & dysfunction  endocardial fibroelastosis (EFE)  backwards flow across PFO  LV stops growing & eventually shrinks

  34. HLHS

  35. NORMAL FETAL 4-CHAMBER

  36. Case Presentation • Term infant born via SVD • Uncomplicated labor and delivery • APGARs of 8 at 1min., 9 at 5min. • Tachypnea noted at 12hrs of life.

  37. Case Presentation • “Airway-Breathing-Circulation” • Respiratory rate (60-90 bpm) • Work of breathing (no retractions) • Saturations (80%) • Warm extremities; good cap refill

  38. Case Presentation No obvious dysmorphic features. More Cardiac Exam Findings: • No murmur. • Single second heart sound (S2). • Hyperdynamic precordium.

  39. Case Presentation • Urgent Cardiology Consult 404-256-2593!! • Cardiac History & Physical • Echocardiogram

  40. Echocardiogram HLHS

  41. Hypoplastic Left Heart Syndrome

  42. Case Presentation • BUT: • No beds available at Egleston immediately • Need to manage infant for 24 hours before transport • NOW what do we do?

  43. Case Presentation • Intravenous access • UVC (double lumen) • UAC • PIV • PIC Remember: AVOID groin lines

  44. Case Presentation • Prostaglandins • 0.03 mcg/kg/min • Side effects • Apnea • Options ? • Intubate vs nasal cannula air

  45. Case Presentation • Labs • Arterial (or venous) blood gas • Electrolytes (normalize) • CBC • LFT • Genetics • Lactic acid • Head and Renal ultrasound • ECHO/EKG

  46. Case Presentation • R/O Sepsis • If no clinical suspicion or maternal indicators no need to start antibiotics • Follow ABG frequently (Q 4 hrs) • Monitor urine output • Monitor for acidosis • Watch for hypotension

  47. Blood Pressure • Blood pressure - systolic and diastolic blood pressures are equally important…not just mean!! • Coronary flow to heart dependant on diastolic BP

  48. Case Presentation • Saturations 95% • pO2 50 • Decreased urine output • Metabolic acidosis • Rising lactic acid What’s going on?!?

More Related