An updated natural history of pre tricuspid shunts.

1. Natural history of Pre-Tricuspid
Shunts
Dr Abhishek Rathore MD DM
Sri Jayadeva Institute of Cardiovascular Science and

2. Pre-Tricuspid Shunts
 Atrial septal defects
 PAPVC
 TAPVC
 RSOV to RA
 Gerbode defect
 Coronary arterial fistula to RA

3. ASD
 8-10% of CHD in children
 30% of CHD in adults
 8-10% risk in offspring of women with sporadic
ASD.
 Incidence- 100/100,000 live births
 OS-ASD (75%)- F:M 2:1
 OP-ASD (20%)- F:M 1:1
 SV-ASD (5%) - F:M 1:1
 CS-ASD (<1%)

4. Natural history of ASD
 Mostly asymptomatic
 May remains undiagnosed until later life
 Detected d/t –abnormal auscultatory finding
- Abnormal ECG/Echo/CXR
- CVA/ HF/ Arrythmia / Pul HTN (esp in
Adults)
 HF
 Recurrent RTI
 Failure to Thrive- Very rare
Moss and Adams 8th edition-Page 676

5.  Dyspnoea on Exertion *
 30% by 3rd decade
 75% by 5th decade
 Supraventricular Arrythmias (AF, AFl)-Prevalance**
 <1 % by 40 yrs
 15% in 40-60 yrs
 60% in >60 yrs
 Mortality**
 After 4th decade – 6% per year
*Braunwald’s Heart disease 10th edition, Page
**Moss and Adams 8th edition-Page 685

6.  Spontaneous closure rate:
Radzik et al JACC 1993 (Mean Age of 28 days)
Mean follow up- 14months:
 < 3mm – 100% close
 3-5 mm – 87% close
 5-8 mm – 80% close
 > 8mm – None close
 Conclusion- Initial ASD diameter was the main
predictor of spontaneous closure.

7. ] Cockerham et al*.Am J Cardiol 1983
 Spontaneous closure rates:
 22% in <12 months old
 33% in 1–2 years old
 3% in 2–4 years old
*Cockerham JT et al. Spontaneous closure of OS-ASD in infants and young children. Am J Cardiol 1

8. ÖZÇEKER D et al, J Kartal TR 2016
Prognosis for Pediatric Patients with Isolated ASD
 Conclusion: Patient age and size of defect at diagnosis
are the most important factors with regard to
spontaneous closure

9. ASD with Eisenmengerisation
 Incidence- 6%
 Mean age of Eisenmengerisation 35 yrs
 Mean age of death 36 yrs
 MCC of death- Hemoptysis (29%)>Surgical closure
(26%)>CCF (17%)
 10 yr survival 80%
Wood P. Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. BM

10. Post surgical closure of ASD
 Independent predictors for long term survival-
 Younger age at operation
 Lower preoperative PAP
 Sx in childhood- usually symptom free, but rarely
can have atrial arrythmias and SSS.
 Sx in 3rd decade and beyond- can have
arrythmias/ Cardiac failure/ stroke/ Pulmonary
vascular disease.
Moss and Adams 8th edition-Page 686

11. Post surgical closure
 Murphy JG et al
 123 pts at Mayo Clinic between 1956 and 1960
 Mean age 26yrs (2-62yrs)
 27-32 yr follow up

12. Atrial Septal Defect
Survival Curves

13. Atrial Septal Defect
Survival Curves

14. Summary
 28 deaths
 13 (48%) Cardiac death
 5 (19%) CVA (all in afib)
 6 (21%) Noncardiac (cancer, sepsis, resp fail)
 Repair < age 24 had normal survival,
 B/w age 25-41 good survival but less than expected,
 > Age 41 had substantial increase in mortality

15. Atrial Septal Defect
 First study to show benefit of surgery in older pt
with ASD
 Retrospective, 179 pt with >40 age (41-79yrs)
between 1966-1991
 47% surgery 53 % medical
 Mean followup of 8.9 ± 5.2 years

16. Medical Surgery
10yr Surv. 84% 95% p=.02
NYHA worse 34% 11%
NYHA better 3% 32%
Afib/flutter 17% 15%
69% improvement in NYHA III/IV

17.  NYHA class III or IV, PASP > 40 mm Hg, and a Qp:Qs
ratio > 2.5:1 at the time of diagnosis were found to be
significant independent predictors of death.
 31% reduction in mortality among symptomatic pt , age >
40 with surgical repair
 Symptomatic improvement in NYHA class and less
deterioration among surgically treated pt
 No effect on atrial arrhythmias
1Konstantinides, et al. Circulation 1994

18.  Rahimtoola et al. Circulation 1968
 Peak PAP >60mmHg had poor surgical outcomes
 Dave et al. Am J Cardiol 1973
 Mean PAP > 40mmHg had poor surgical
outcomes.

19. Outcomes after Device closure
 N= 236
 Conclusion-At any age, ASD closure is followed
by symptomatic improvement and regression of
PAP and RV size.
 However, the best outcome is achieved in patients
with less functional impairment and less elevated
PAP.

20. ASD Device closure
 Meta-analysis of 142 case series-
 Major periprocedural complications – 1.6%
 Device embolisation necessitating surgery (0.7%) and
pericardial tamponade (0·1%) being the m.c.
 MC minor complications
 atrial arrhythmias, vascular complications, and transient
heart block.
Late complications
 Atrial arrhythmias (1·5%),
 stroke (0·4%),
 device thrombosis(0·2%),
 device erosion through the atrial wall or aortic root (0·1%),
 Device embolisation (0.1%),

21. Regression of cardiomegaly
 Most of the decrease in RA/RV size occurs
immediately within 1–2 years later.
 High likelihood of normalisation of RV size- if
Young age and less RV size pre-ASD closure.
 Persistent enlargement of the right heart has
been reported in up to a third of patients, mostly
in adults with severely dilated chambers
Du ZD et al. Speed of normalization of right ventricular volume overload after
transcatheter closure of ASD in children and adults. Am J Cardiol 2001; 88: 1450–53.

22. OP-ASD
 M=F
 Physiological consequences and longevity is similar
to OS-ASD of equivalent size. (without MR and Pul
vascular disease)
 If non-restrictive OP-ASD with severe left AV valve
regurgitation- CHF occurs and mortality 33% in first
year.
 Associated Downs syndrome influence longevity bec
of accelerated pul vascular disease.
 Referred for surgical repair between 1 and 4 years of
age, slightly younger than for secundum ASD, often
due to the more pronounced dilation of right sidedPerloff’s Clinical recognition of congenital heart disease. 6th

23. OPERATION FOR PARTIAL
ATRIOVENTRICULAR SEPTAL DEFECT: A
FORTY-YEAR REVIEW
 N=334 at Mayo clinic 1955-1995
 30-day and 5-, 10-, 20-, and 40-year survival were
98%, 94%, 93%, 87%, and 76%, respectively
 Closure of the left AV valve cleft and age < 20 years
at operation were associated with better survival
 Reoperation was performed for 38 patients (11%)
 Repair of residual/recurrent left AV valve regurgitation
or stenosis was the most common reason for
reoperation
 The median interval between the primary repair and
the subsequent reoperation was 17.8 yearEl-Najdawi et al. OPERATION FOR PARTIAL ATRIOVENTRICULAR SEPTAL DEFECT: A FORTY-YEAR REVIEW.
The Journal of Thoracic and Cardiovascular Surgery 2000:Volume 119, Number 5

24.  LVOTO occurred in 36 patients and 7 patients
underwent reoperation to relieve this obstruction.
 Supraventricular arrhythmias were observed in 58
patients (16%) after the operation.
 Complete AV block occurred in 9 patients (3%).
 Permanent pacemakers were implanted in 11
patients.

25. Common Atrium
 Cyanotic malformation of non-restrictive ASD
 Ellis van crevald syndrome- a common association.
 Systemic O2 is often >90%
 Symptoms and PHTN begin early and more pronounced.
 In 1st year of life- Dyspnea/fatigue/respiratory
infections/mild cyanosis/ physical underdevelopment very
common.
 Occasionally, patients relatively well till early adolescence.
But rare in adulthood.

26. PAPVC
 Less than four pulmonary veins connected to RA or
its tributaries.
 M=F
 Associations
Asplenia and polysplenia
Turner and Noonan
 MC type of PAPVC is to RSVC-RUPV
 2ND MC is to RA

27. Natural history -PAPVC
 Fundamental physiologic disturbance is similar to
ASD
Factors determining hemodynamic state-
 No of anamolous connected veins
 Cross-sectional area of veins
 Presence/absence and size of ASD

28.  PAPVC with intact IAS
 If single PV is anomalously connected- Rarely
recognised and has excellent prognosis.
 If all but one of PVs drain anamolously-
Physiology and clinical features comparable to
TAPVC
 PAPVC with ASD
 Similar to ASD

29.  During 3rd and 4th decade- Frequency of patients
presenting with cyanosis increase d/t Pul HTN
and increasing Rt to Lt shunt.
 Post surgical outcomes- similar to post ASD
closure.
Moss and Adams 8th edition-Page 819

30. Scimitar Syndrome
 3-6% of PAPVC
 75% have isolated form---- benign outcome
 25% have other CHD ---- increase CHF and
mortality

31. No correlation was found between patient age and the presence of significant r
hypoplasia and the presence of symptoms at diagnosis
Vladimiro L et al. Rev Esp Cardiol. 2013;66:556-60 - Vol. 66 Num.07

32.  Median follow-up was 6.4 years (range, 0.2-27.5
years). None of the patients underwent correction
of scimitar vein drainage.
 Two patients, both with associated CHDs died of
HF
 First died at the age of 14 months from CHF and
severe untreatable PHT and the second died at
the age of 5 months from CHF, pulmonary
hypertension and acute renal failure after surgical
correction of a left anomalous pulmonary venous
drainage.

33.  Conclusion: Majority of patients are asymptomatic as
isolated lesion. If associated CHD present, high risk of
CHF,PHT and increase mortality.

34. TAPVC
 Incidence: 4 to 6 per 1,00,000 live births.
 2% of deaths due to CHD in first year of life
 Prognosis in TAPVC depends on:
1. Size of interatrial communication
2. Presence of obstruction to pulmonary venous
drainage

35. TAPVC without pulmonary venous
obstruction
 M=F
 Asymptomatic at birth.
 Tachypnea and feeding difficulties- usually in first
few weeks.
 Followed by frequent RTI and FTT.
 Cyanosis is mild because of adequate mixing.
Moss and Adams 8th edition-Page 828
Northern Great Plains Registry of Congenital Heart
Disease
N=74
56% symptoms in first month, remainder in first year

36.  Cardiac failure in most patients prior to 6 months
of age.
(Hepatomegaly is always present and peripheral
edema is present in about half of the cases).
 75-85% die by 1year of age, most in first 3
months of life.

37. TAPVC with pulmonary venous
obstruction
 M>F
 Tachypnea, tachycardia and cyanosis within few
days of life.
 If left untreated, death may occur from pulmonary
edema and RVF within 2days to 4.5 months.
Moss and Adams 8th edition-Page 83
Northern Great Plains Registry of Congenital Heart
Disease(1981)
N= 43 patients
72% symptoms in first month, remainder early in first
year

38.  Post-operative course:
 N= 768 (2005-2014)
 Operative mortality 5%
 9 year survival was 91%
 Surgical outcomes poor if-
 younger age at repair , infracardiac and mixed
anatomic variation, pre-operative PVO, longer
duration of CPB and ventilation.
Guocheng Shi et al. Total Anomalous Pulmonary Venous Connection: The Current Management Stra
Cohort of 768 Patients Circulation. 2017;135:48-58

39. RSOV to RA
 1% of CHD
 RCC (80%) > NCC (20%)
 80% of SOVA rupture
 Mean age of rupture – 34 yrs
 Gradual rupture or unruptured aneurysm may go
unnoticed.
 Unruptured SOVA- 20 yr survival is 95%
 Ruptured SOVA- once symptomatic, die within 1yr if
unoperated.
 MCC of death is HF>IE
 Surgical mortality <2%.

40. Gerbode defect
 LV to RA communication
 M:F 2.2:1
 3 types by Sakakibara and Konno:
 Type I (Supravalvular) 76%- LV to RA across membranous
septum
 Type II (infravalvular) 16%- VSD with TR to RA
 Type III (Mixed) 8% - Combined
 Congenital (rare)
 Acquired- Iatrogenic/ IE/Trauma/MI
 Spontaneous closures are less likely in type I LV-RA shunt but
 more in acquired than in congenital shunts

42.  VSD size d/t traumatic LV-RA shunt > iatrogenic or an
infective LV-RA shunt
 Interventionally treated patients had the highest
postinterventional complications ie,33% vs 12%
 Interventionally managed patients- More residual
shunts
 Surgically treated patients- more frequent
postoperative heart blocks.
 Surgically managed patients showed the highest
survival ie,76%
 Non-intervention patients had the highest mortality
rate,43.3%.
Shi-Min Yuan. Hellenic J Cardiol 2015; 56: 357-372