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Harvard Medical SchoolBoston Children’s Hospital
Multi-Organ Dysfunction Syndrome & Pulmonary Hypertension
Amy Sanderson, MD
Boston Children’s Hospital
Harvard Medical SchoolBoston Children’s Hospital
Case example
• 10 year old male with ALL undergoing chemotherapy
• Presented to the ED with febrile neutropenia
• Exam:
– Lethargic and hard to arouse
– Hypotensive and poorly perfused
• Labs:
– Creatinine 2
– AST & ALT 200-300
– INR 1.8
• Fluid resuscitated & pressors initiated
• Intubated
Harvard Medical SchoolBoston Children’s Hospital
The ICU team documents that he is admitted for septic shock and multi-organ
dysfunction
Harvard Medical SchoolBoston Children’s Hospital
MODS
• Why do clinicians use the term?
– Easy to explain how sick a patient is
• What’s the problem with the term
– Non-specific
– Can’t code it
Harvard Medical SchoolBoston Children’s Hospital
What is MODS?
• Clinical syndrome of otherwise unexplained physiologic dysfunction of > 1 organ systems
• It is a continuum of physiologic derangements
– Ranges from mild to irreversible failure
• Induced by a variety of acute insults
– Sepsis, pancreatitis, cardiac arrest, hemorrhagic shock
Harvard Medical SchoolBoston Children’s Hospital
Pathophysiology
• Unregulated immune response
• Uncontrolled, severe inflammation
• Release of inflammatory mediators/cytokines
• Inadequate perfusion/tissue hypoxia
Harvard Medical SchoolBoston Children’s Hospital
Respiratory Dysfunction
• Pulmonary vascular endothelial injury
– Disrupted capillary blood flow
– Enhanced microvascular permeability
– Pulmonary edema
• Neutrophil entrapment within the pulmonary microcirculation
– Injury to alveolar capillary membranes
• ALI & ARDS
Harvard Medical SchoolBoston Children’s Hospital
Respiratory Dysfunction
• Exam: dyspnea, tachypnea, nasal flaring, hypopnea
• Data: O2 sat with hypoxia, blood gas with hypoxia &/or hypercapnia
• Treatment: oxygen, positive pressure
• Documentation: respiratory insufficiency, desats
Harvard Medical SchoolBoston Children’s Hospital
Respiratory dysfunction
• What should clinicians document?
–Acute (on chronic) respiratory failure
–Acute pulmonary edema
– Pleural effusion
– Pneumothorax
Harvard Medical SchoolBoston Children’s Hospital
Circulatory Dysfunction
• Vasodilation: inappropriate distribution of blood flow
– More blood flow to periphery
– Ischemia of core organs
– Tachycardia
• Pre-existing heart disease may not be able to tolerate increased metabolic demand
Harvard Medical SchoolBoston Children’s Hospital
Circulatory Dysfunction• Systolic & diastolic cardiac dysfunction
– Both pre-existing cardiac disease and not
• Mitochondrial dysfunction – end organs cannot extract oxygen sufficiently
• Hypotension
– Endothelial injury: capillary leak/hypovolemia
– Vasodilation: decreased venous return
– Cytokine release: myocardial depression
Harvard Medical SchoolBoston Children’s Hospital
Circulatory Dysfunction
• Exam: tachycardia, hypotension, delayed or flash capillary refill, cool & mottled skin or flushed skin
• Data: metabolic acidosis, ↑ lactate, ventricular dysfunction
• Treatment: IV fluid boluses, vasoactive infusions
• Documentation: low BP, soft BPs
Harvard Medical SchoolBoston Children’s Hospital
Circulatory Dysfunction
• What should clinicians document?
–Hypotension (at the least!)
–Metabolic acidosis
–Heart failure (with specificity)
– Shock (septic, cardiogenic)
Harvard Medical SchoolBoston Children’s Hospital
Gastrointestinal Dysfunction
• Overgrowth of bacteria in upper GI tract: aspiration pneumonia
• Alteration of gut mucosal barrier: translocation of bacteria (worsening sepsis)
• Decreased gut motility from sepsis, opioids, paralytics: impaired nutritional intake
• Liver dysfunction: synthetic, transaminitis
Harvard Medical SchoolBoston Children’s Hospital
Gastrointestinal Dysfunction
• Exam: abdominal distention, RUQ tenderness
• Data: ↑bili, AST & ALT, distended bowel loops on KUB
• Treatment: IV fluid bolus, bowel rest, TPN, methylnaltrexone, laxatives, stool softener
• Documentation: transaminitis, low albumin, weight loss
Harvard Medical SchoolBoston Children’s Hospital
Gastrointestinal Dysfunction
• What should clinicians document?
– Aspiration pneumonia
– Ileus
– Shock liver / ischemic hepatitis
– Malnutrition
– Hypoalbuminemia
Harvard Medical SchoolBoston Children’s Hospital
Renal Dysfunction
• Decrease in intravascular volume
• Hypotension
• Renal vasoconstriction
• Release of inflammatory mediators/cytokines
• Activation of neutrophils by endotoxins and other peptides
Harvard Medical SchoolBoston Children’s Hospital
Acute Tubular Necrosis
MedicationsHypovolemia
Low cardiac output states
Systemic vasodilation
DIC
Ischemic
Myoglobin & hemoglobin
Toxic
Crystals
Multiple myeloma
Harvard Medical SchoolBoston Children’s Hospital
Acute Tubular Necrosis
Initiation
• Hypotension & hypovolemia
• Ischemia
• Injury/death of tubular cells
• Sloughing & cast formation
• Obstruction of tubular lumen
• Cytokines
Maintenance
• Stabilization of GFR at a very low level
• Lasts 1-2 wks
Recovery
• Regeneration of tubular cells
• Sometimes polyuria
• Salt & H2O loss
Harvard Medical SchoolBoston Children’s Hospital
Renal Dysfunction
• Exam: delayed capillary refill, cool & mottled extremities, thready pulses, AMS, oozing
• Data: ↑ BUN & Cr, ↑K & Phos, ↑/↓ Na, ↓HCO3
• Treatment: IV fluid bolus
• Documentation: renal insufficiency, azotemia, requiring IHD or CVVHD, diuretics
Harvard Medical SchoolBoston Children’s Hospital
Renal Dysfunction
• What should clinicians document?
– Acute kidney injury/failure
• With ATN, if present
– Underlying CKD (with stage)
Harvard Medical SchoolBoston Children’s Hospital
Coagulation Defects
• Ranges from mild coagulopathy to DIC
• Exam: bleeding, bruising, purpura fulminans
• Data: ↑INR, PT & PTT, ↓ fibrinogen, ↑D-dimer, ↓ PLT
• Treatment: PLTs, FFP, cryoprecipitate
• Documentation: abnormal coags, prolonged INR
Harvard Medical SchoolBoston Children’s Hospital
Coagulation Defects
• What should clinicians document?
– Coagulopathy
– DIC
– Thrombocytopenia
– Anemia (acute blood loss)
Harvard Medical SchoolBoston Children’s Hospital
MODS
• Clinical syndrome of otherwise unexplained physiologic dysfunction of > 1 organ systems
• It is a continuum of physiologic derangements
– Ranges from mild to irreversible failure
• Induced by acute insults
• Goal is to treat dysfunction of individual organ systems as well as the underlying cause
Harvard Medical SchoolBoston Children’s Hospital
Case example
• Newborn female with a pre-natal diagnosis of left CDH
• Intubated at birth
• ABG with a significant respiratory and metabolic acidosis.
• Echocardiogram:
– Moderate RV systolic dysfunction
– Moderate PDA with flow going across the PDA to the aorta
– Supra-systemic PA pressures
Harvard Medical SchoolBoston Children’s Hospital
The ICU team documents that the patient is started on inhaled nitric oxide due to
elevated PA pressures
Harvard Medical SchoolBoston Children’s Hospital
What is pulmonary hypertension?
• Complex, progressive disease affecting both children and adults
– Leads to significant morbidity and mortality
• Mean pulmonary artery pressure ≥ 25mmHg when the patient is at rest
Hansmann G. J Am Coll Cardiol. 2017 May 23; 69(20): 2551-2569
Oishi P, Fineman JR. Pediatr Crit Care Med. 2016 Aug; 17(8 Suppl 1): S140-5
Harvard Medical SchoolBoston Children’s Hospital
WHO classification
• Group 1 - PAH
• Group 2 - PH due to left-sided heart disease
• Group 3 – PH due to lung diseases and/or hypoxia
• Group 4 - Chronic thromboembolic PH
• Group 5 – PH with unclear or multifactorial etiologies
Simonneau G, et al. J Am Coll Cardiol. 2009 Jun 30. 54(1 Suppl):S43-54
Harvard Medical SchoolBoston Children’s Hospital
Causes of pulmonary hypertensionCongenital heart disease
Lung disease/chronic hypoxia (BPD, COPD, OSA, interstitial lung disease)
Idiopathic
Heritable
Persistent PH of the newborn
Congenital diaphragmatic hernia
Glycogen storage disease
Connective tissue disease
Chronic pulmonary embolism
Ischemic left heart disease
Cardiomyopathy
Myocarditis
Drug-induced (chemotherapy, cocaine)
Hematologic disorders (chronic hemolytic anemia, myeloproliferative disorders, splenectomy)
Chronic renal failure
Portal hypertensionSimonneau G, et al. J Am Coll Cardiol. 2009 Jun 30. 54(1 Suppl):S43-54
Harvard Medical SchoolBoston Children’s Hospital
Pathophysiology
Vasoconstriction
ThrombosisRemodeling
Harvard Medical SchoolBoston Children’s Hospital
Pathophysiology
High resistance in the pulmonary vascular bed
Blood flow obstructed from the RV to the lungs
Increased blood volume & pressure in RV (↑work!)
Over time, the RV dilates and thickens
RV dysfunction & failure
Harvard Medical SchoolBoston Children’s Hospital
PH crisis
↑↑ RV pressure and volume
Interventricular septum shifts to the left
LV’s ability to fill is impeded
↓ cardiac output & systemic oxygen delivery
Hypoxia, metabolic acidosis & possibly death
Harvard Medical SchoolBoston Children’s Hospital
Signs & symptoms
• Dyspnea with exertion
• Fatigue
• Lethargy
• Syncope
• Cyanosis
• Anorexia & weight loss
• Cough
• Chest pain
• RUQ pain
Harvard Medical SchoolBoston Children’s Hospital
Exam
• Systolic ejection murmur over L sternal border
– May be louder during inspiration
• 4th heart sound (S4): “Tennessee”
• RV failure
– Hepatomegaly
– Ascites
– Peripheral edema
– 3rd heart sound (S3): “Kentucky”
Harvard Medical SchoolBoston Children’s Hospital
Diagnostic studies
• PA catheter readings
• Echocardiogram
• Chest CT
• Cardiac MRI
• Cardiac catheterization
Harvard Medical SchoolBoston Children’s Hospital
Therapies
• Oxygen
• Pulmonary vasodilators
• Inotropic/vasoactive agents
• Diuretics
• Calcium channel & beta blockers
• Anticoagulation
• Bicarbonate
• Sedation & paralysis
• Surgical therapies
Harvard Medical SchoolBoston Children’s Hospital
Documentation
• Elevated PA or wedge pressure
• RV strain
• RV hypertrophy
• RV dysfunction
• Pulmonary vasoreactivity
• Nitric oxide responder
Harvard Medical SchoolBoston Children’s Hospital
Pulmonary Hypertension
• Complex, progressive disease affecting both children & adults, leading to significant morbidity and mortality
• There are many causes of PH
• There may be many clues in the medical record, including history, exam, diagnostics, therapies & documentation that may point to the diagnosis of PH