CONGESTIVE CARDIAC FAILURE IN NEWBORNSDR ANITA S

DEPT. OF NEONATOLOGY,KEMGH,PAREL,MUMBAI

ETIOLOGY OF CONGESTIVE CARDIAC FAILURE

The neuro-humeral response that eventually results in the variable symptom complex known as heart failure is elicited when the hemodynamic demands on the heart exceed the flow generating capacity of the systemic pump.

Due to limited inflow/outflow Limited inflow in lesions like mitral

stenosis,pulmonary venous obstruction,restrictive cardiomyopathy,pericardial disease

Due to limited outflow-dilated cardiomyopathy, systemic outflow obstruction.

Volume overload lesions-when limited diastole capacity is accompanied by limited systolic capacity-shunt lesions and regurgitant lesions

When normal hemodynamic demands are imposed on myocardium with diminished contractile capacity

When excess load is imposed on normal myocardium

In a case of CCF in newborn it is important to know

Presence or absence of congenital heart disease

Presence or absence of myocardial dysfunction.

Age of presentation

In newborn myocardial dysfunction is rare. Structural problems that were silent in utero

due to parallel circulation and high pulmonary vascular resistance often present after birth in response to the normal neonatal fall in the pulmonary vascular resistance.

The right ventricle through the ductus arteriosus can help supply systemic blood flow which is then affected by the normal closing of the ductus in the postnatal life.

Etiology of CCF in the structurally normal heart

PRENATAL ANAEMIA ARRYTHMIA AV FISTULA CARDIOMYOPATHY TWIN TO TWIN

TRANSFUSION

NEONATES AND INFANTS ANAEMIA ARRYTHMIA AV FISTULA DILATED CARDIOMYOPATHY ENDOCRINOPATHIES HYPOGLYCAEMIA HYPOTHYROIDISM HYPOXIC-ISCHAEMIC INJURY HYPERTENSION SEPSIS HYPOCALCEMIA

ETIOLOHY OF CCF IN PATIENTS WITH CONGENITAL HEART DISEASE

PRENATAL ATRIOVENTRICULAR

VAVE REGURGITATION MITRAL STENOSIS

WITH INTACT ATRIAL SEPTUM

NEONATE AND INFANTS SYSTEMIC INFLOW

OBSTRUCTION COR TRIATRIATUM MITRAL STENOSIS PULMONARY VENOUS

STENOSIS SYSTEMIC OUTFLOW

OBSTUCTION AORTIC VALVE STENOSIS COARCTATION OF AORTA SUBAORTIC STENOSIS TRUNCAL VALVE STENOSIS

SYSTEMIC VENTRICULAR VOLUME OVERLOAD

AORTIC/MITRAL REGURGITATION

PDA ASD,VSD TAPVC TRNCUS ARTERIOSUS AV CANAL DEFECT SINGLE VENTRICLE

PATHOPHYSIOLOGY

MOST MEDICAL THERAPIES FOR TREATMENT OF CCF ARE BASED ON INTERRUPTING THE REFLEX NEUROHUMERAL MECHANISMS THAT ACCOUNT FOR MANY OF THE SYMPTOMS OF HEART FAILURE

THE BASIC EVENT IS INADEQUATE CARDIAC OUTPUT.THE CARDIOVASCULAR SYSTEM HAS NO FLOW SENSOR TO PROVIDE DIRECT FEEDBACK TO THE HEART THAT MORE FLOW IS REQUIRED.

AT THE TISSUE AND REGIONAL LEVELS DECREASE IN BLOOD FLOW LEADS TO INCREASE IN LOCAL METABOLITE CONCENTATIONS WHICH LEADS TO LOCAL VASODILATION AND INCREASED FLOW

THE DECREASED PERIPHERAL RESISTANCE LEADS TO FALL IN BLOOD PRESSURE,SIGNALS ARE SENT TO SYSTEM-LEVEL ARTERIAL VASORECEPTORS WHICH LEADS TO ACTIVATION OF THE SYMPATHETIC NERVOUS SYSTEM(SNS) AND RENIN-ANGIOTENSIN-ALDOSTERONE SYSTEM(RAAS).

ACTIVATION OF THE SYMPATHETIC NERVOUS SYSTEM LEADS TO

TACHYCARDIASTIMULATION OF MYOCARDIAL

CONTRACTILITYREGIONAL VASOCONSTRICTION

ACTIVATION OF THE RAAS LEADS TO RENAL FLUID RETENTION

WHICH LEADS TO INCREASED VASCULAR VOLUME.THIS

IMPROVES CARDIAC FILLING AND RESTORES CARDIAC OUTPUT

THROUGH UTILIZATION OF THE PRELOAD RESERVE.

THESE FINELY TUNED MECHANISMS PRESERVE THE FLOW AND

VASCULAR VOLUME IN NARROW LIMITS DESPITE SIGNIFICANT

HEMODYNAMIC PETURBATION.

HOWEVER WHEN THE FALL IN THE CARDIAC OUTPUT AND

SECONDARY FALL IN BLOOD PRESSURE ARE DUE TO DIMINISHED

CARDIAC CAPACITY,UTILIZATION OF THE PRELOAD RESERVE MAY BE

INADEQUATE TO INCREASE THE CARDIAC OUTPUT WITHOUT

INTOLERABLE RISE INTHE CIRCULATING VOLUME AND FILLING

PRESSURE.

UNDER THESE CIRCUMSTANCES,THE SYMPTOM COMPLEX OF HEART

FAILURE WHIHCH IS PRIMARILY A MANIFESTATION OF SYSTEMIC AND

PULMONARY VENOUS HYPERTENSION ENSUES.

ULTIMATELY HEART FAIURE IS A COMPROMISE BETWEENTHE

SYMPTOMS ASSOCIATED WITH INADEQUATE CARDIAC OUTPUT AND

SYMPTOMS ASSOCIATED WITH VENOUS HYPERTENSION.

CHRONIC ACTIVATION OF THE COMPENSATORY

MECHANISMS EXACERBATES THE DISORDER.

CONSEQUENCES OF CHRONIC VOLUME EXPANSION ARE

WELL-KNOWN –PULMONARY OEDEMA,PULMONARY

HYPERTENSION.PERIPHERAL OEDEMA AND GI

DYSFUNCTION.

LESS OBVIOUS IS THE CAPACITY FOR THESE MECHANISMS

TO DAMAGE THE MYOCARDIUM DIRECTLY.

ALDOSTERONE HAS BEEN SHOWN TO CONTRIBUTE DIRECTLY

TO MYOCYTE APOPTOSIS AND INCREASE THE RISK OF

VENTRICULAR ARRYTHMIAS AND SUDDEN DEATH.

CHRONIC ACTIVATION OF THE SNS LEADS TO REDUCTION IN

BETA-ADRENERGIC RECEPTOR DENSITY AND NORADRENALINE

RESERVES LEADING TO DIMINUTION OF ADRENERGIC

INOTROPIC RESERVE .

THE CHRONIC ELEVATION IN FILLING PRESSURES LSTIMULATES

VENTRICULAR REMODELLING,INCREASING DIASTOLIC

CAPACITANCE AT THE EXPENSE OF INCREASED SYSTOLIC WALL

STRESS AND INCREASED MYOCARDIAL OXYGEN CONSUMPTION.

BOTH THE SNS AND RAAS STIMULATE GENERALIZED VASOCONSTRICTION AND REDISTRIBUTION OF BLOOD FLOW MAINTAINING THE BLOOD SUPPLY OF THE VITAL ORGANS AT THE EXPENSE OF CUTANEOUS,MUSCULAR AND RENAL BLOOD FLOW.

SKELETAL MUSCLE UNDERPERFUSION CAUSES

ANAEROBIC METABOLISM AND LACTIC ACIDOSIS

WHICH FURTHER LEADS TO SYMPTOMS OF EFFORT

INTOLERANCE AND FATIGUE.

REDUCTION IN THE RENAL BLOOD FLOW LEADS TO

ELECTROLYTE DISTURBANCES AND NITROGEN

RETENTION.

IN ADDITION TO THE ACTIVATION OF THE RAAS AND SNS WITH

THE COUNTER-REGULATORY NATRIURETIC PEPTIDE ,THERE IS

ALSO AUGMENTED RELEASE OF VASOPRESSIN AND

ENDOTHELIN,PEPTIDE GROWTH FACTORS(E.G. TGF-

BETA),INFLAMMATORY CYTOKINES(E.G. TNF-ALPHA,IL-1BETA).

THESE WITH OTHER LOCAL FACTORS ARE BELIEVED TO PLAY

A ROLE IN THE VASCULAR REMODELLING AND MYOCARDIAL

REMODELLING.

Assessment - History

MATERNAL HISTORY AGE

ADVANCED AGE- RISK OF DOWN’S SYNDROME W/ASSOCIATED ENDOCARDIAL CUSHION DEFECT

COMORBIDITIES DIABETES- POSSIBILITY OF VENTRICULAR SEPTAL HYPERTROPHY LUPUS- RISK OF COMPLETE HEART BLOCK

MEDICATIONS/DRUGS VALPROIC ACID- ASSOCIATED WITH COA, HLHS, AS

ILLNESS RUBELLA- PDA

FAMILY HISTORY PREVIOUSLY AFFECTED CHILDREN?

Assessment - History

PREGNANCY HISTORY

FETAL ULTRASONOGRAPHY RESULTS

KNOWN GENETIC DEFECT OR SYNDROMIC FEATURES

ANY COMPLICATING FEATURES (EG OLIGO- OR

POLYHYDRAMNIOS)

BIRTH HISTORY

GESTATIONAL AGE

MODE OF DELIVERY

APGARS, TRANSITION, RESUSCITATION NEEDS

SYMPTOMS OF CCF IN NEONATES

FEEDING DIFFICUTIES ARE THE MOST PROMINENT SYMPTOM IN

NEONATES WITH CCF.

AS FEEDING IS THE MOST PHYSICALLY DEMANDING ACTIVITY IN

NEWBORNS,CCF LEADS TO PROLONGED FEEDING

TIMES,TACHYPNOEA,TACHYCARDIA AND PERSPIRATION.

THE EFFORT OF SUCKING AND MAINTAINING A HIGH RESPIRATORY RATE

WITH THE DIVERSION OF BLOOD FLOW TO THE GUT WHILE FEEDING

TAXES THE LIMITED CARDIAC RESERVE.

HENCE GROWTH IS THE BEST MEASURE OF SEVERITY OF CCF IN

INFANTS.

PHYSICAL FINDINGS

TACHCARDIA(>160/MIN IN NEONATES AND >120/MIN IN INFANTS)

TACHYPNOEA(>60/MIN IN NEONATES AND >40/MIN IN OLDER INFANTS)

FURTHER VENTILATORY COMPROMISE-NASAL

FLARING,RETRACTIONS,GRUNTING

WHEEZING DUE TO COMPRESSION OF THE AIRWAYS BY DISTENDED

HYPERREACTIVE PULMONARY VESSELS.

RALES ARE NOT COMMON IN INFANTS WITH CCF ,MAY INDICATE

COEXISTING PNEUMONIA.

AUSCULTATION OF THE CRANIUM AND LIVER FOR BRUIT SHOULD BE DONE.

DYSMORHIC FEATURES SHOULD BE LOOKED FOR.

CARDIAC FINDINGS-

A QUIET PRECORDIUM MAY BE SEEN IN CARDIOMYOPATHIES.

PROMINENT CARDIAC LIFT IN SHUNT LESIONS.

SYSTOLIC THRILL IN OBSTRUCTIVE LESIONS

THIRD HEART SOUND MAY BE HEARD BUT IT IS DIIFICULT TO APPRECIATE

IN PRESENCE OF TACHYCARDIA.

DISTENSION OF NECK VEINS MAY BE APPRECIATED IN OLDER INFANTS.

HEPATOMEGALY IS A VALUABLE PHYSICAL SIGN OF HEART FAILURE.

PERIPHERAL OEDEMA IS NOT COMMON IN INFANTS AND IT IS SEEN IN

VERY SEVERE HEART FAILURE.

COOL EXTREMITIES,WEAK PULSES,LOW BLOOD PRESSURE AND

NARROW PULSE PRESSURE ARE SEEN AS MANIFESTATIONS OF

LOW CARDIAC OUTPUT.

MOTTLING AND DELAYED CRT ARE INDICATIVE OF MORE

SEVERE VASCULAR COMPROMISE.

MODIFIED ROSS HEART FAILURE CLASSIFICATION FOR CHILDREN

CLASS I

ASYMPTOMATIC

CLASS II

MILD TACHYPNEA OR DIAPHORESIS WITH FEEDING IN INFANTS

DYSPNEA ON EXERTION IN OLDER CHILDREN

CLASS III

MARKED TACHYPNEA OR DIAPHORESIS WITH FEEDING IN INFANTS

MARKED DYSPNEA ON EXERTION

PROLONGED FEEDING TIMES WITH GROWTH FAILURE

CLASS IV

SYMPTOMS SUCH AS TACHYPNEA, RETRACTIONS, GRUNTING, OR DIAPHORESIS AT

REST

INVESTIGATIONS-CHEST X RAY

CHEST XRAY IN NEONTES WITH CCF ALMOST ALWAYS

DEMONSTRATES CARDIOMEGALY EXCEPTION BEING

LEFT ATRIAL OBSTRUCTIVE LESIONS LIKE COR

TRIATRIATUM AND OBSTRUCTED TAPVC.

EXCESSIVE PULMONARY BLOOD FLOW WITH DIFFUSE

HAZINESS.

INCREASED LUNG VOLUMES WITH FLATTENED

DIAPHRAGMS.

INVESTIGATIONS-ECG

USE OF ECG IS NOT FOR DIAGNOSIS OF CCF,IT MAY PROVIDE VALUABLE CLUES IN IDENTIFYING UNDERLYING LESION.

INVESTIGATIONS-2D ECHO

TO IDENTIFY THE STRUCTURAL HEART DISEASE,ASSESSMENT

OF VENTRICULAR FUNCTIONS,PERICARDIAL

EFFUSIONS,SEVERITY OF MITRAL REGURGITATION WHICH MAY

ACCOMPANY CCF.

THE DIAGNOSTIC APPROACH IN A PATIENT WITH NEW ONSET HEART

DISEASE DEPENDS UPON THE AGE,PRESENCE OF CONGENITAL

HEART DISEASE AND ASSOCIATED COMORBIDITIES.

ECG,CXR AND ECHO ARE MANDATORY.

DIAGNOSIS MAY BE DIFFICULT IN THE ABSENCE OF STRUCTURAL

DEFECT.

24 HOUR HOLTER MONITORING,EVALUATION FOR

MYOCARDITIS,SEARCH FOR REVERSIBLE CAUSES SUCH AS

ENDOCRINOPATHIES,METABOLIC DISORDERS AND MITOCHONDRIAL

DISORDERS SHOULD BE UNDERTAKEN.

INVESTIGATION-CARDIAC CATHETERIZATION

TO RULE OUT STRUCTURAL ABNORMALITIES SUCH AS

ANOMALOUS CORONARY ARTERIES, AV MALFORMATIONS.

IN SOME SELECTED SITUATION A COMBINED MYOCARDIAL

BIOPSY MAY BE WARRENTED.

MANAGEMENT

ACUTE CHF

IN EMERGENCY PATIENT TO BE STABILIZED

MAINTAINANCE OF THERMO-NEUTRAL ENVIRONMENT

ELEVATION OF HEAD END OF COT.

RYLE’S TUBE FEEDING.

CORRECTION OF ANAEMIA.

TREATMENT OF FEVER.

CORRECT ACIDOSIS WITH FLUID RESUSCITATION OR SODIUM BI-

CARBONATE APPROPRIATELY.

TREAT HYPOCALCEMIA.

THRESOLD FOR CALCIUM ADMINISTRATION SHOULD BE LOWER

IN CONDITION SUCH AS TRUNCUS ARTERIOSUS AND

INTERUPTED AORTIC ARCH.

ABSENT FEMORAL PULSES OR INABILITY TO INCREASE THE

SYSTEMIC ARTERIAL PaO2 TO ABOVE 150 mm Hg IN HYPEROXIA

TEST SUGGEST A DUCTAL DEPENDENT LESION AND TREATMENT

WITH PGE1 IS WARRANTED.

TREATMENT OF ARRYTHMIAS WITH D/C CARDIO-VERSION OR

MEDICAL THERAPY.

INTRA-VENOUS IONOTROPIC SUPPORT SHOULD BE INITIATED IN

LOW OUTPUT STATES.

MEDICATION ACTION DOSAGE

1 DOPAMINE β1,DA1 5-28 MCG/KG/MIN IV

2 DOBUTAMINE β1 5-28 MCG/KG/MIN IV

3 MILRINONE PHOSPHODIASTERASE INHIBITOR

0.25-0.75 MCG/KG/MIN IVLOAD-50 MCG/KG IV OVER 15 MIN

4 EPINEPHRINE α AND β 0.05-1 MCG/KG/MIN

CHRONIC CHF

GENERAL MEASURES:MAINTAINANCE OF ADEQUATE

NUTRITIONAL INTAKE WITH CALORIE DENSE

FEEDINGS.CONSULTATION MAY BE REQUIRED WITH

NUTRITIONIST

MAINTAINANCE OF GOOD NUTRITION CORRELATES WITH

IMPROVED SURGICAL OTCOMES.

CONSIDERATION OF GASTROSTOMY FEEDINGS/NASOJEJUNAL

TUBES.

FLUID RESTICTION

SODIUM RESTRICTION.

DIURETICS INCREASE SODIUM AND WATER EXCRETION EITHER BY

INCREASING RENAL BLOOD FLOW OR INHIBITING ABSORPTION OF NaCl

IN THE TUBULES, THUS INCREASING URINARY VOLUME

FUROSEMIDE IS THE MOST COMMONLY USED DIURETIC IN PAEDIATRICS.

IT ACTS ON THE LOOP OF HENLE INHIBITING REABSORPTION OF NaCl

and along with it H2O. IT CAN CAUSE PREVENTION OF RESORPTION

NEARLY 25% NaCl.

FEW RCTs ARE AVAILABLE ON USE OF DIURETICS IN CHILDREN BUT

THEY ARE WIDELY USED IN VIEW OF SYMPTOMATIC RELIEF THEY

OFFER.

IF GIVEN INTRAVENOUSLY THERE IS A PROMPT AND IMPRESSIVE

RESPONSE PROVIDED THERE IS A GOOD CARDIAC OUTPUT.

ADVERSE EFFECTS INCLUDE ELECTROLYTE IMBALANCES LIKE

HYPONATREMIA,HYPOKALEMIA,METABOLIC ALKALOSIS.

ACE INHIBITORS

AS MENTIONED EARLIER, THE ACTIVATION OF SNS AND RAAS

ARE ACUTELY BENEFICIAL BUT IN THE LONG TERM THEY

CONTRIBUTE TO THE PROGRESSION OF CCF.

ACEIs DISRUPTS THE ACTIVATION OF RAAS AND IS BENEFICIAL

IN HEART FAILURE.

IN ADULTS, LARGE CLINICAL TRIALS HAVE SHOWN THAT

THERAPY WITH ACEIs IMPROVES SYMPTOMS AND INCREASE

SURVIVAL.

EXPERIENCE WITH ACEIs IN CHILDREN INCLUDES SEVERAL

SMALL OBSERVATIONAL STUDIES IN CHILDREN WITH

CARDIOMYOPATHIES OR VOLUME OVERLOAD SECONDARY TO

SHUNT LESIONS.

MONTIGNY AND COLLEGUES FOUND THAT A SINGLE DOSE OF

CAPTOPRIL IN 12 INFANTS WITH VSD CAUSES DECREASE

SYSTEMIC VASCULAR RESISTANCE, DECREASE PULMONARY TO

SYSTEMIC FLOW RATIO THROUGH INCREASE IN SYSTEMIC

CARDIAC OUTPUT.

ANGIOTENSION RECEPTOR BLOCKERS

EXPERIENCE IS LIMITED IN CHILDREN

RESERVED FOR THOSE PATIENTS WHO CANNOT TOLERATE

ACEIs.

DIGITALIS

THE PRINCIPLE EFFECT ON MYOCARDIUM IS TO INCREASE THE

FORCE AND VELOCITY OF CARDIAC MUSCLE CONTRACTION.

THIS INOTROPIC EFFECT IS DEPENDENT ON VARIOUS IONS SUCH

AS SODIUM,POTASSIUM,CALCIUM,MAGNESIUM AND IS

INDEPEDENT OF ADRENERGIC STIMULATION.

IT INHIBITS THE Na-K ATPase PUMP ,WHICH MAINTAINS HIGH

INTRACELLULAR CONCENTRATION OF SODIUM.

THIS ALTERS THE EXCITATION-CONTRACTION COUPLING

MAKING AVAILABLE LARGER AMOUNTS OF INTRACELLULAR

CALCIUM INCREASING TH FORCE OF CONTRACTION.

FOR CHILDREN WITHVMYOCARDIAL FAILURE THIS APPEARS TO IMPROVE THE CARDIAC OUTPUT.

NO EVIDENCE ON WHETHER THERE IS IMPROVED SURVIVAL IN CHILDREN WITH MYOCARDIAL DYSFUNCTION ON DIGITALIS.

SOUND THEORETICAL BASE AND YEARS OF EXPERIENCE DICTATE ITS USE IN THIS SITUATION,

IN CHILDREN WITH CCF DUE TO LARGE SHUNT LESIONS THE BASIS FOR USE IS LESS CLEAR AS THE MYOCARDIALFUNCTION IS GENERALLY GOOD IN THESE SITUATIONS.

EFFICACY RELATES TO ELECTRO-PHYSIOLOGICAL PROPERTIES AND INCREASE IN SENSITIVITY TO ARTERIAL BARORECEPTOR REFLEX,LEADING TO INCREASED VAGAL TONE AND DECREASES ADRENERGIC TONE LEADING TO REDUCTION IN RESTING HEART RATE.

DRUGS FOR CHRONIF HEART FAILURE

DRUG ACTION DOSAGE

1 DIGOXIN Na-K ATPase INHIBITION

PREMATURE 15 MCG/KG/DAY,INFANT <10KG 10MCG/KG/DAY

2 FUROSEMIDE LOOP DIURESIS 1MG/KG/DOSE PO OR IV MAY INCREASE QID

3 ACEIs(CAPTOPRIL) INHIBITION OF ACE 0.1-0.5 MG/KG/DAY PO 8 HOURLY

4 POTASSIUM SUPPLEMENT

5 BETA BLOCKERS BETA1 SELECTIVE (METOPROLOL) AND NON-SELECTIVE BETA BLOCKAGE

0.1-0.5 MG/KG/DOSE, INCREASE UPTO 1.1MG/KG/DOSE

REFERENCES

BLUME E.D. et al, NADA’S PAEDIATRIC CARDIOLOGY 2ND EDITION, 76-89

SCHOLZ T.D. et al, AVERY’S DISEASE OF THE NEWBORN 9TH EDITION, 774-786

SUNDARARAGHAVAN S, IAP SPECIALITY SERIES OF PEDIATRIC CARDIOLOGY, 56-61