Basics of pediatric anomalies of heart, a practical perspective.

1. Congenital Heart Disease & Surgical
Management
Rakesh Kumar Sharma
(Cardio Thoracic Nursing)
Nursing Officer,
CTVS-OT Safdarjung Hospital,
New Delhi

2. Introductions:-
A congenital heart defect (CHD) or Congenital heart anomaly
or Congenital heart disease, is a problem in the structure of the heart
that is present at birth. Signs and symptoms depend on the specific
type of problem.
It is responsible for 3-5% of death during first week of life and up to 33% of
death during the entire neonatal period.

3. History of Pregnancy Especially 3 Months:
 Viral infection Rubella(PDA,VSD), CMV, Coxsackie virus(acute myocarditis)
 Drug exposure: Exposure to cardio toxic drugs( ACE inhibitors,
Antidepressants/antipsychotics, Anxiolytics and Lithium, Beta-blockers,
Cytotoxics, NSAIDs, Antiepileptics, Opioids, Ratinoids, Salicylates, Sex hormoness,
Sulphonamides, Tetracyclines, Warfarin/Coumaeins
 Alcohol: fetal alcohol syndrome (VSD, ASD)
 Maternal disease: Diabetes(VSD, TGA, Cardiomyopathy), SLE(Comp. AV Block)
 Exposure to Radiation
2% CHD are environmental in origin
Hereditary & Non-Hereditary Syndromes associated with cardiovascular anomalies
Family history of CHD (If mother 8%, sibling 5%, Syndrome in parents like marfan
syndrome 8%) along with still birth and spontaneous abortion.
 BIRTH HISTORY, BIRTH WEIGHT, GENDER
ETIOLOGICAL FACTORS:-

4.  Irritability & Lethargy
 Tachypnea
 Palpitation
 Dyspnea
 Cyanosis
 Feeding difficulties and failure to thrive
 Inadequate growth
 Sweating and pallor
 Exercise tolerance
 Arrhythmias
 Chest pain
 Congestive Heart Failure: - (It occurs in 30% of children
with CHD among these 90% occurs in infants)
 Sudden death
Identification of CHD in infant & children:-
Clubbing Fingers

5. CYANOSIS: -
 Central cyanosis - (Physiologic up to 20 mts after birth) Around the core, lips, and tongue.
Ex: TOF
 Acrocyanosis - (Persistent bluish discoloration of extremities, Cardiopulmonary/Pathologic)
 Differential cyanosis – (Cyanosis more prominent in lower extremities. Ex-PDA)
 Cutis marmorata – (Purple marble like mottling of skin due to exposure to cold)
Paroxysmal Hypoxemic spell: -(Cyanotic attack with quick audible breath with visible distress)
More frequent in infancy and spontaneous improvement 18m-2yr
Squatting position: - (It increases systemic vascular resistance, reduces Rt-Lt shunt, improves
pulmonary flow, reduces heart rate.

6. Diagnosis:-
The systemic approach to CHD includes:
Detailed antenatal and postnatal history.
Systemic physical examination: General
Examination followed by detailed examination of
the cardiovascular system by Inspection, Palpation,
Percussion & Auscultation. The examination of
Respiratory system & Abdomen should be done.
Electrocardiography (ECG)
Chest X-Ray
Echocardiography (Antenatal 18-22weeks, After
birth)
Computed Tomography (CT) Angiogram
Cardiac Magnetic Resonance Imaging (MRI)
Invasive procedures: Cardiac Catheterization &
Angiocardiography

11. CHD with Increased Pulmonary Blood Flow:-
 Patent ductus arteriosus – PDA
 Atrial Septal Defect – ASD
 Ventricular Septal Defect – VSD
 Aorot-Pulmonary window
 Truncus Arteriosus
 D-Transposition of Great Arteries - D-TGA
 Partial/Total Anomalous Pulmonary Venous
Return : P-APVR/T-APVR

12. Patent Ductus Arteriosus: PDA
Sx:- PDA Ligation

13. Atrial Septal Defect- ASD: Four types
1. Ostium primum
2. Ostium secundum
3. Sinus venosus
4. Coronary sinus
Sx: ASD Patch closure

14. Ventricular Septal Defect- VSD: Types
1. Perimembranous VSD
2. Subpulmonary VSD
3. Muscular VSD
4. Atrio-Ventricular canal
Sx: - VSD Patch Patch Closure

15. Aorto-Pulmonary Window-AP Window: 3 Types
Sx: - Arch Anomalies Correction & Patch Closure of Defect

16. Atrio-Ventricular Canal: AV Canal:-
Sx: - A.V. Canal Repair wt ASD/VSD Patch Closure &
A.V. Valve Reconstruction.

17. Truncus Arteriosus:-
Sx: - Truncus Repair or Rastelli Operation

18. D-Transposition of Great Arteries: D-TGA
Sx: - Arterial Switch Procedure

19. Partial/Total Anomalous Pulmonary Venous Return:- P-APVR/T-APVR:
Sx: - Re-Routing of TAPVC or Anastomosis of the Common Pulmonary Veins
to Left Ventricle & ASD/PDA Closure

21. CHD with Decreased Pulmonary Blood Flow:-
 Pulmonary stenosis
 Tetrology of fallot – TOF
 TOF with Pulmonary Atresia
 Tricuspid Atresia
 Double Outlet Right Ventricle

22. Tetrology of Fallot - TOF
Sx:-Intra Cardiac Repair or Total Correction: Pulmonary Valvotomy
/ Trans Annular Patch + VSD closure with RVOT Reconstruction

23. Tricuspid Atresia: Three stages of surgery
1. Modified B.T. Shunt. 2. Bidirectional Glenn Shunt. 3. Fontan Procedure

24. Duble Outlet Right Ventricle: DORV
Sx: Intra Ventricular tunnel btw VSD & Sub Aortic Outflow Tract

25. CHD with Decreased Systemic Blood Flow:-
 Coarctation of Aorta – COA
 Interrupted Aortic Arch
 Aortic Stenosis
 Hypoplastic Left Heart Syndrome
 Ebstein’s Anomaly

26. Sx:- End to End Anastomosis or Occasionally Graft to Patch Aorta
Coarctation of Aorta :-COA

27. Interrupted Aortic Arch:
Sx: Aortic Arch Reconstruction with or without Patch Augmentation

28. Aortic stenosis:
Sx:- AS-Valvotomy/AS wt Insufficency-Valvuloplasty/Bicuspid Valve-Ross
procedure / Sub Aortic stenosis-Konno procedure
Ross Procedure

29. Hypoplastic Left Heart Syndrome:
Sx: - 1st Norwood stage first,
2nd Bidirectional Glenn Shunt-BDG,
3rd Fontan procedure

30. Ebstein’s Anomaly:
Sx:- Tricuspid Valvuloplasty / T.V.
Replacement

31. Palliative Procedures:-
1. Pulmonary Artery Banding – PA
Banding
2. Blalock-Tausing Shunt – BT Shunt
3. Bi-Directional Glenn Shunt – BDG
4. Norwood
Norwood stage 1st & 2nd

32. Corrective Repair:-
 Rastelli
 Damus Kaye Stansel
 Fontan
 Mustard
 Senning
 Rashkind Balloon Septostomy
 Atrial Septostomy
 Ross Procedure
 Konno Procedure

33. Rastelli Procedure:-
Intra ventricular repair & Placement of Extra Cardiac RV to PA Conduit.
Done in DORV with Sub Pulmonary stenosis/D-TGA +VSD & LVOTO

34. Damus Kaye Stansel:-DKS
First stage palliative procedure for Single Ventricle with Sub Aortic
Stenosis.
 Single Left Ventricle
 Trans Position of Great Arteries: TGA

35. Fontan Procedure:- Three types
1. Atrio-Pulmonary connection
2. Extra Cardiac Total Cavo-Pulmonary Connection
3. Intra Cardiac Total Cavo-Pulmonary Connection
It is the final Reconstructive procedure for Single Ventricle

36. Mustard & Sennings Procedure:
Diversion of Systemic & Pulmonary Blood. Done in TGA

37. Rashkind Procedure- Balloon Atrial Septostomy:

38. Ross Procedure:
Alternative to Prosthetic valve replacement for Aortic stenosis or
Insufficiency

39. Konno Procedure:
Done for Valvular or Subaortic Stenosis/ Annular narrowing of LVOT

40. Congenital Cardiac Conditions:-
 C-TGA
 Anomalous Coronary Artery
 Cardiac Malposition: Levocardia, Dextrocardia, Mesocardia & Ectopia Cardis
 Situs discordance between organ systems
 Shones Complex: Anatomic collection of multiple Lt sided obstructive lesions
 Hereditary & Non-Hereditary Syndromes associated with cardiovascular
anomalies

41. L-Transposition of Great Arteries: L-TGA/C-TGA

42. Anomalous Coronary Artery: ALCAPA
Re-implantation of Left Coronary Artery in to the Aortic root.
Pulmonary Trunk
Left Coronary
Lengthning

43. Cardiac Malposition, Situs Discordance:-

44. Nursing Assessment
Active participation in plan of medical care
 Obtain thorough Nursing history of the child.
 Discuss with physician the Plan for medical care. Permote Team work.
Make a Baseline Nursing Assessment of the child’s condition
 Observe and record child’s
1. Growth & development.
2. Exercise tolerance level.
3. Color and Temperature changes of Skin/Mucous membrane
4. Clubbing of the fingers, Thickening of terminal phalanges
5. Chest deformities.
6. Respiratory pattern.
7. Pulses in all extremities.
8. Auscultate the child’s heart & Record Vital signs.
Roll of Nursing Officer in Management of Congenital Heart Disease:

45. Nursing Interventions
 Provide Adequate Nutritional & fluid intake.
 Prevent infection
 Reduce the workload of the heart.
 Observe child for Symptoms of Congestive Heart Failure.
 Observe for Symptoms of infective endocarditis.
 Observe for the development of thrombosis.
 Prepare the child for diagnostic and treatment procedures.
 Nursing counseling & Discussion.

46. Health Education
 Encourage for Necessary measures to maintain the child’s health.
 Educate family about defect & associated plan.
 Encourage to treat child in a normal manner.
 Parents participation & its importance in Nursing care.
 Follow up plans.

49. Thank