the cardiac circle

8/10/2019 the cardiac circle

1/75

1

THE CARDIAC CYCLE


2/75

2


3/75

3


4/75

4


5/75


6/75

6


7/75

7

Irsad Andi Arso


8/75

8

Overview

US: 1,000,000 adults with congenitalheart dz20,000 more patients reachadolescents yearly

*All figures from ACCSAP V unless otherwise noted


9/75

9

Adult Congenital Heart Disease

Atrial Septal DefectVentricular Septal Defect

Patent Ductus ArteriosusCoarctation of AortaTetralogy of Fallot

Ebstiens Anomaly


10/75

10

Hole between the two atriaBlood flows left to right

PFO

Patent foramen ovale fails tocloseRight heart becomes dilated

Too much blood to the lungs


11/75

11

Three types

Primum ASDSecundum ASD

Sinus venosusAVSD Atrio ventricular septaldefect


12/75

12

Atrial Septal Defect1/1500 live birthsSecundum most common ACHD (6-10%) RAD

Primum associated with other endocardial cushion defects (cleft AV

valves, inlet type VSD) LAD

Sinus Venosus large, associated with anomalous pulmonary venous drainage(usually R superior PV)

Coronary sinus (rare) associated with unroofed coronary sinus


13/75


14/75

14


15/75

15

Physiologic Consequences

Shunt Flow Size of defect Relative compliance of ventricles Relative resistance of pulmonary/systemic circulation

L R shunting results in diastolic overload of RV andincreased pulmonary blood flowRV dilatation/failure and rarely severe pulm HTN(Eisenmengers) may ensue over time ~5%

With age, deterioration chiefly due to1

decrease LV compliance, increased L R shunt increase in atrial arrhythmias pulm HTN develops, RV volume + pressure OL

1Perloff, NEJM 1995


16/75

16

Clinical Symptoms Often asymptomatic until 3-4 th decade formoderate-large ASD, may present later inlife for initially smaller ASD Fatigue DOE

Atrial arrhythmias Paradoxical Embolus Recurrent Pulmonary infections


17/75

17

Physical SignsS2 wide/fixed splitting RV/PA palpable impulse (if lg defect)

systolic ejection murmur 2nd

L ICS mid-diastolic TV rumble


18/75

18

Auscultation in ASDIncreased flow across the pulmonaryvalve produces a systolic ejection murmurand fixed splitting of the second heart soundFixed splitting of S2 may in part be due todelayed right bundle conduction.Increased flow across the TV produces adiastolic rumble at the mid to lower rightsternal border.

Older pt loses pulm ejectionmurmur as shunt becomesbidirectional

signs of pulm HTN/ CHF maypredominate


19/75

19

ECG


20/75

20

ECHO

Subcostal

view of

Intraatrial

SeptumColor Flow/

Contrast

Good for

secundum,primum


21/75

21

TreatmentMedical : diuretics, ACEI, AldactoneRepair

Consider when sxs, Qp:Qs>1.5 Interventional (Percutaneous Closure)

Only for secundum defects94-96% success (Amplatzer) adequate superior/inferior rim around ASD no R-L shunting


22/75

22

Surgical Closure Good prognosis:

closure age < 25, PA pressure 25 or PA>40, decreased survival due toCHF, stroke, and afib

Treatment


23/75

23

Hole between the two ventriclesLeft to right shunt majorityDilated right heart too much blood tolungs increase in pulmonary pressureSmaller defects can closespontaneously


24/75

24


25/75

25

Three types

Perimembranous VSD most commonMuscular VSD can be multiple

Apical VSD

usually smallVariable in size


26/75

26


27/75

27

Ventricular Septal Defect

May be anywhere inintra-ventricular septum-clinical course dependson the shunt size and

involvement ofpulmonary vascular bed.Approx of all VSDs aresmall, and more than close spontaneously.

Highest closure rates in thefirst decade of life.


28/75

28

PATHOPHYSIOLOGY

primarily depends on size&status of pulm. vascular bed rather thanlocationSmall communication (less than 0.5cm`) VSD is restrictive &rt.ventricular pressure is normal does not cause significanthemodynamic derangement(Qp:Qs=1.75:1.0)Moderately restrictive VSD with a moderate shunt(Qp:Qs=1.5-2.5:1.0)&poses hemodynamic burden on LVLarge nonrestrictive VSDs(more than 1.0cm`) Rt&Lt ventricular pressureare equalised(Qp:Qs is more than 2:1)Large VSDs at birth ,PVR may remain higher than normal and Lt to Rtshunt may intially limited involution of media of smallpulm.arterioles,PVR decreases large Lt to Rt shunt ensuesIn some infants large VSDs ,pulm. arteriolar thickness never decreases

pulm.obstructive disease develops .when Qp:Qs=1:1 shunt becomesbidirectional,signs of heart failure abate &pt. becomes cyanotic.(Eisenmenger syndrome)


29/75

29

CL INICA L FEA TURES

Race : no particular racial predilectionSex :no particular sex preferenceAge :

infants difficult in postnatal period,although ccf

during first 6mths is frequent,X-ray&ECG arenormal. children after first year variable clinical picture

emerges.small VSD asymptomaticlarge VSD:-palpitation,dyspnoea on exertion,feeding

difficulties ,poor growth-frequent chest infections


30/75

30

PHYSICA L FINDINGS

Pulse pressure is relatively wide

Precordium is hyperkinetic with a systolic thrill at LSBS1&S2 are masked by a PSM at Lt.sternal border,max. intensity of the murmur is best heard at3 rd ,4 th &5 th Lt interspace.Also well heard at the 2 nd

space but not conducted beyond apexLt. 2 nd space widely split &variable accentuated P2Delayed diastolic murmur at the apex &S3Presence of mid-diastolic ,low pitched rumble at theapex is caused by increased flow across the mitralvalve


31/75

31

INVESTIGATIONS

CHEST RADIOGRAPHY

- normal- biventricular hypertrophy- pulmonary plethora

ELECTROCARDIOGRAPHY

-smallVSD ~ normal tracing-mod.VSD ~ broad,notched P wave characteristic of Lt. Atrial overload aswell as LV overload,namely,deep Q waves & tall R waves in leadsV5 and V6 and often AF-large VSD ~RVH with rt. axis deviation. With further progressionbiventricular hypertrophy;P waves may be notched/peaked.


32/75

32

ECHOCARDIOGRAPHYtwo-dimensional &doppler colour flow

ANGIOGRAPHY(cardiac catheterization and angiography )

INVESTIGATIONS .


33/75

33

COMPLICATIONS

Congestive cardiac failureInfective endocarditis on rt.ventricular side

Aortic insufficiencyComplete heart blockDelayed growth & development (FTT) in infancyDamage to electrical conduction system during

surgery(causing arrythmias)Pulmonary hypertension


34/75

34

INTERVENTION

3 MAJOR TYPESSMALL (less than 3mmdiameter)- hemodynamically

insignificant- b/w 80-85% of all VSDs- all close spontaneously

* 50% by 2yrs* 90% by 6yrs* 10% during school yrs

- muscular close soonerthan membranous


35/75

35

MODERATE VSDs * 3-5mm diameter* least common group of children(3-5%)* w/o evidence of ccf/ pulm.htn can be

followed until spontaneous closure

occurs. LARGE VSDs WITH NORMAL PVR

* 6-10mm in diameter* usually requires surgery Conservative

treatment


36/75

36


37/75

37

DEFINITIONPatent ductus arteriosus(PDA) is a heart problemthat is usually noted inthe first few weeks ormonths after birth. It ischaracterized by aconnection between theaorta and the pulmonaryartery, which allowsoxygen-rich (red) bloodthat should go to thebody to re-circulatethrough the lungs


38/75

38

IN DEPTH


39/75

39

IN GROSS


40/75


41/75

41

HEMODYNAMICS

LARGE FLOW THRULT VENT-DELAYEDCLOSURE OF

AORTIC VALVEDILATATION OFASCENDINGAORTA

LATE A2PARADOXICALLYSPLIT S2

AORTIC EJECTIONCLICK

AORTIC EJECTIONSYSTOLICMURMUR


42/75

42

PRESENTATION

Frequent chest infectionsfatiguesweatingrapid breathingheavy breathingcongested breathing

disinterest in feeding, or tiring while feedingpoor weight gain


43/75

43

On examination

Inspection Carotid pulsations Hyperkinetic & lt

ventricular type ofapical impulse

Palpation Systolic or

continous thrill at2 nd lt interspace


44/75

44

AuscultationAccentuated s1Narrowly or paradoxically split s2

Loud p2Continous/gibsons/train-in-tunnelmurmur best heard in infraclavicularregionMitral delayed diastolic murmur


45/75

45

CXR FINDINGS

CARDIOMEGALY

LA ENLARGEMENTLV ENLARGEMENTPROMINENTAORTIC KNUCKLEPULM PLETHORA


46/75

46

ECG CHANGES

NORMAL AXIS WITH LTVENTRICULAR HYPERTROPHY

ECHO PICTURES


47/75

47

ECHO PICTURES


48/75

48

MANAGEMENT

MEDICAL INDOMETHACIN 0.1

mg/kg/dose,orally,bd for three doses Digoxin for increasing working capacity

of heart Diuretics to reduce preload on heart


49/75

49

SURGICAL REPAIR :DEVICES


50/75

50


51/75

51

Coarctation of the Aorta

Males twice as frequently as females.98% of all coarctations at segment ofaorta adjacent to ductus arteriosus.Produced by both an externalnarrowing and an intraluminalmembrane.Blood flow to the lower bodymaintained through collateralvessels.


52/75

52

Narrowing in

proximal descendingaortaMay be long/tubularbut most commonly

discrete ridgeNatural hx: poorprognosis ifunrepaired

AorticAneurysm/dissection CHF Premature CADz


53/75

53


54/75

54

CLINICAL

Absent or weak femoral pulses.Systolic pressure higher in upperextremities than in lowerextremities; diastolic pressures aresimilar.Harsh systolic murmur heard in theback.Rib notching on CXR pathognomonic


55/75

55

Rib notching


56/75

56

TreatmentDespite surgery, patients still have significantmorbidity/mortality with average age 38

Up to 70% of repaired patients still go on to developHTN, pathology not well understood

Recurrence in 8-54% of repairs, can undergo repeatsurgery or balloon angioplasty

Aortic Aneurysm/ruputure may occur despite

successful repair and correction of HTN (freqaround anastomosis site on patch repair 30% inone study)


57/75

57


58/75


59/75

59


60/75

60


61/75

61


62/75

62


63/75

63


64/75

64


65/75

65


66/75

66

ToF: Surgical Treatment

Systemic Pulmonary Shunt Blalock-Taussig Waterston (RPA)

Potts (LPA)Complete Repair

takedown of prior shunt patch VSD resection of subpulmonic obstruction transannular patch around pulm valve annulus

(usually leads to severe PI)

Ebsteins Anomaly


67/75

67

Ebsteins Anomaly

Atrialization of RV, sail-likeTV, TR50% ASD/PFO50% ECG evidence ofWPWAge at presentation variesfrom childhood adulthoodand depends on factorssuch as severity of TR,Pulm Vascular resistancein newborn, andassociated abnormalitiessuch as ASD

www.ucch.org


68/75

68

Massive cardiomegaly,mainly due to RAE


69/75

69

Ebsteins: Clinical Presentation

Pediatric murmur

Adult (unrepaired with ASD) atrial arrhythmias murmur cyanosis

R L shunt NOT due to PulmHTN but TR jet directed

across ASD exercise intolerance

Surgery in pts with significant TR/sxs


70/75

70

Eisenmengers Syndrome

Final common pathway for allsignificant L R shunting in whichunrestricted pulmonary blood flowleads to pulmonary vaso-occlusivedisease (PVOD); R Lshunting/cyanosis devleops

Generally need Qp:Qs >2:1


71/75

71


72/75

72


73/75

73

Eisenmenger Complications

Coagulopathy/platelet consumptionBrain abcessesCerebral microemboliAirway hemorrhage

especially moving from lower higheraltitudes (air travel, mountains)


74/75


75/75

the cardiac circle

Documents