Tetralogy of fallot by Dr Maheen Ihsan..pptx

Tetralogy of fallot is common Pediatric congenital heart disease, it has 4 components
1)Ventricular septal defect
2)Over-riding aorta
3)Right ventricular hypertrophy
4)Right ventricular outflow tract obstruction
Right ventricular outflow tract obstruction is the dynamic component of TOF , and increase knee severity of RVOTO is responsible for characteristic hypercyanotic spells, also known as tet spells.
Right ventricular outflow tract obstruction is the dynamic component of TOF , and increase knee severity of RVOTO is responsible for characteristic hypercyanotic spells, also known as tet spells.
Chest X-Ray shows classi Boot shaped heart , and Electrocardiography shows evidence of Right axis deviation with Right ventricular hypertrophy.
Echocardiography is diagnostic.
Treatment is via complete surgical correction as early as 4-6months of life. In children, where co-morbid conditions donot permit complete surgical closure, a palliative BT-shunt may be performed.
Prognosis is excellent in case of surgical correction, while uncorrected shunts may lead to complications such as cerebrovascular accidents , cerebral abcess , infective endocarditis and growth failure.

1. Dr Maheen Ihsan PGR Pediatrics-II Tetralogy of Fallot

2. Congenital heart disease affects 8-9 / 1000 live births, and approximately 25% are considered CCHD. The incidence of CHD increase to 2% to 6% for a second pregnancy after the birth of a child with CHD or if a parent is affected. It is estimated that 35% of infant deaths due to congenital malformations are related to cardiovascular anomalies. Congenital Heart Disease

3. Estimated prevalence for congenital cardiac anomalies in Pakistani population is 11/1000. Do pakistani babies have more congenital heart defects? Preliminary findings from our birth cohort study P. Agadoorappa 1 , et al. Archives of Disease in Childhood, Volume 96. Disease burden in Pakistan

4. Factors associated with risk of CHD

5. Congenital heart disease Cyanotic Acyanotic ⬆️PBF ⬇️PBF L--> R shunt Obstructive Miscellaneous TOF Tricuspid Atresia VSD with PS TGA TAPVR Persistent truncus Arteriosus ASD VSD PDA HLHS Critical AS Interrupted aortic arch Infantile type Co-Aorta Aortic stenosis Pulmonary stenosis Congenital MR

6. L—> R SHUNT

7. Tetralogy of fallot

8. 1672 1777 1888 1924 Stenson-published a brief paper titled “Dissection of a Monstrous Foetusin Paris.” Eduard Sandifort, a Dutch physician, reported a case in a 16-month-old patient called “the blue boy. Etienne-Louis Arthur Fallot described the classical tetrad. He used the terms, “La maladie bleue” (the blue disease) or “cyanose cardiaque” (cardiac cyanosis). Maude Elizabeth Abbott-entitled it “tetralogy of Fallot.” Tetralogy of Fallot

9. 4-5 per 10,000 live births. 7-10% of congenital heart diseases. Most common cynaotic congenital heart disease presenting after first month of life. Equal incidence in Males and Females. Epidemiology

10. Multi-factorial. Non-Syndromic :- NKX-2 transcription factor. MTHFR polymorphism. Sydromic (15%):- Down syndrome. Allagile Syndrome. Di-George/ Velocardiofacial syndrome. Genetics

11. Components Right ventricular outflow tract obstruction Large VSD. Overriding aorta Concentric RV hypertrophy

12. Does TOF has increased or decreased pulmonary blood flow.

13. Clinical Presentation

14. Clinical Presentation (Age at presentaction) Depends upon degree of RVOT obstruction Severe Mild-moderate Minimal Profound cyanosis in newborn period Asymptomatic initially As degree of RVOT worsens Cyanosis and Tet spells appears May even develop heart failure d/ t unrestricted blood flow. Asymptomatic initially

15. Hyper cyanotic spell /tet spell Caused by infandibular spasm Characterized by:- Periods of uncontrollable cry/panic. Rapid and deep breathing. Deepening of cyanosis. Decreased intensity of heart murmur. may progress to loss of consciousness & cardiac arrest. Peak Age:- 2-24 months. Risk factors:- Vigorous cry, Excercise, dehydration.

16. Complications CNS Brain Abcess (>2years of age) Cerebrovascular accident (<2years of age) CVS Infective endocarditis MAPCAs Hematological Polycythemia Relative Iron deficiency. MSK Gout Arthropathy General Growth failure

17. Clinical Examination General Observation:- Dysmorphism :- Down syndrome, Digeorge syndrome. Anthropometry :- Height , Weight, OFC-Failure to thrive; small and short for age. General Physical Examination:- Hands :-Cyanosis , Clubbing. Splinter hemorrhages , Osler Nodes , Janeway lesions-signs of Infective endocarditis. Pulse :- Usually normal. High volume:-associated PDA. Absent Unilateral pulse:- classic BT-shunt. Conjunctiva :- Hyperemia. Mouth :- Central cyanosis, Oral hygiene. Gum hypertrophy. Edema :-Congestive Cradiac Failure.

18. Which one is painful

19. Cardiovascular Examination Inspection :- Precordial bulge :- RV-hypertrophy. Scar Marks:- Lateral throacotomy:- BT-shunt. Midline Sternotomy:- Corrective surgery. Palpation :- Apex beat-Normal position and character. Left Parasternal heave- Right Ventricular hypertrophy. Auscultation :- S1 + S2 + Ejection systolic murmur at left upper sternal border. Clinical Examination

20. Is murmur in TOF d/t VSD or RVOT obstruction?

21. Why does murmur disappear in Tet spell?

22. Gastrointestinal Examination Spleenomegaly:- Infective Endocarditis. Hepatomegaly:- Congestive Cardiac Failure. Bulk , Tone , Power , Deep tendon Reflexes Nervous system Examination Bulk, Tone, Power , Deep Tendon Reflexes, Babinski Sign , Gait Analaysis— Brain Abcess / Cerebrovascular accident. Clinical Examination Musculoskeletal Examination Joiint swelling- Small joint arthropathy.

23. Investigations

24. Chest Xray Minimal to no cardiomegaly, Concavity of left heart border. Oligemic lung fields. Up-lifted apex d/t RVH.

25. Normal CXR CXR in TOF

26. ECG Right axis deviation QRS axis >90. Two lead method:- QRS axis positive in Lead avF & negative in Lead 1. Right ventriclular hypertrophy. R/S ratio >1 in v1. R-wave in v1 >7mm. Right Atrial Enlargement. P- pulmonary- Tall spiked wave (>2.5mm)-

27. Echocardiography All four components of TOF. Size, number and position of VSD. Degree of Right ventricular outflow tract obstruction. Associated anomalies.

28. Right ventricular angiogram

29. CBC ABGs CRP,Blood C/S MRI/CT scan Polycythemia , Thrombocytopenia, Leukocystosis- Infections Metabolic acidosis If suspected infection/ infective endocarditis If Thromboses/ abcess suspected. Supportive Labs

30. Management

31. Medical Management Neonates presenting with severe RVOT obstruction. Prophylaxis for tet spells while waiting for surgery. Treament of Tet Spell. Treatment of Complications. Indications

32. Neonate with severe RVOT obstruction:- These patients may require intravenous prostaglandin therapy i.e alprostadil (PGE 1; 0.01-0.20 pg/kg/min) to maintain ductal patency and pulmonary flow pending surgical or catheter-based intervention. Beta blockers:- Oral propanolol (0.5-1mg/kg every 6hours) is used to decrease frequency and severity of hypercyanotic spells. Medical Management

33. Calm down the patient . Lose any constrictive clothing . Oxygen inhalation . Knee chest position . Intravenous fluid bolus -Normal saline (15-20ml/kg). Subcutaneous morphine (0.2mg/kg) Intravenous sodium bicarbonate . Intravenous Propranolol (0.1mg/kg). Intrave phenylephrine (bolus dose of 5 to 20 mcg/kg per dose, followed by continuous infusion). Mechanical ventilation Emergency BT-shunting. Management of Tet spells

34. , ' Treatment of Complications Cerebral thromboses / CVA Supportive , Hydration , Phlebotomy. Brain Abcess Intravenous Antibiotics , Surgical drainage Infective endocarditis Intravenous Antibiotics Polycythemia Phlebotomy. Relative iron deficiency Iron replacement Gingival disease Oral hygiene Gout Alluprinol

35. Surgical Management Palliative surgical procedures. Corrective surgical procedures.

36. ●Infants who have severe RVOT obstruction. ●Infants who are too small to undergo complete repair (eg, preterm infants). ●Infants with a medically refractory severe hypercyanotic ("tet") spell. ●Infants with coronary anatomy complicating initial complete repair in the neonatal period. ●Infants with PA hypoplasia who would not tolerate biventricular repair. Palliative Surgical Procedures Indications:- To enhance blood flow from systemic to pulmonary circulation.

37. Named after Alfred Blalock (surgeon to first perform this procedure) and Helen Taussig (pediatric cardiologist, who designed the shunt). 1st performed in a 15-month-old girl with tetralogy of Fallot in November 1944 at Johns Hopkins University Hospital in Baltimore, Maryland (USA). Blalock Taussig Shunt Types:- Classical:- Connects Right subclavian artery to pulmonary artery. Modified:- Uses Gore-Tex conduit. Alfred Blalock, MD, FACS

38. Peri-operative injury to surrounding structures:- Throacic duct—>Chylothorax. Sympathetic trunk—> Horner syndrom. Phrenic nerve—> Diaphragmatic paralysis. Shunt complications:- Thrombosis. Infection. Blockage. Dislodgement. Others:- Heart Failure :- due to large size of shunt. Infective endocarditis. Complications of BT-shunt

39. On August 31, 1954, Clarence Walton Lillehei performed the first successful repair of tetralogy of Fallon at the University of Minnesota. Timing:- Stable patient with no spell:- 4-6 month of age. As soon as 1st cyanotic spell occurs. Not usually performed in neonatal period due to increased risk. In a meta-analysis of eight observational studies, mortality was higher for neonates who underwent early compete repair compared with later repair (6 versus 1 percent, respectively) Loomba RS, et al. Complete Repair of Tetralogy of Fallot in the Neonatal Versus Non-neonatal Period: A Meta-analysis. Pediatr Cardiol. 2017 Jun;38(5):893-901. PMID: 28190140. . Corrective Surgical Procedures :-

40. Pulmonary regurgitation. Tricuspid Regurgitation. Right Heart Failure. Residual VSD. RBBB , as VSD is close to AV-node. Peri-operative mortality:- 0-3% Complications:- Procedure:- Resection of RVOT. Patch closure of VSD. Valvotomy/ valevectomy if associated valvular stenosis

41. Unrepaired TOF is associated with poor survival, with one-half of affected individuals dying in the first few years of life and most uncorrected patients not living beyond the third decade. Repaired TOF:- Long-term survival is excellent following TOF repair. A single center study of 734 patients who underwent TOF repair in early childhood (median age 17 months) between 1986 and 2007 with a median follow-up of 12.5 years, overall survival rates were 95, 93, and 93 percent at 10, 20, and 25 years, respectively. Park CS, Lee JR, Lim HG, Kim WH, Kim YJ. The long-term result of total repair for tetralogy of Fallot. Eur J Cardiothorac Surg. 2010 Sep;38(3):311-7. doi: 10.1016/j.ejcts.2010.02.030. Epub 2010 Mar 25. PMID: 20346688. Prognosis:- Unrepaired TOF :-

42. QUESTIONS???

43. THANK YOU !