Managing VSDs: Specific Challenges in the Low Resource Environment Krishna Kumar AIMS, Cochin India

VSD Common Surgical Correction Rewarding Immediate Intermediate Long-term

LMICs This slide shows the human development indices in various parts of the world. The purpose here is to show the fact that an overwhelming majority of the world’s population lives in regions that have relatively low HDI. The Demic Atlas Project: Toward a Non-State-Based Approach to Mapping Global Economic and Social Development, by Martin W. Lewis, Jake Coolidge, and Anne Fredell Source: http://geocurrents.info/category/demic-atlas#ixzz25BKi757t

Worldmapper Population Cartogram This worldmapper cartogram depicts the area of a region in proportion to population and again demonstrates that the vast majority of the worlds population are in “emerging economies http://www.worldmapper.org/display.php?selected=263#

Estimated proportion of infants and newborns with critical CHD undergoing surgery within the first year of life in India Number undergoing surgery in the first year of life Estimated number of infants with critical CHD 2012

The Indian Situation Barriers to timely care of CHD: Poverty Treatment Ignorance Socio-cultural (gender bias) Geographic Treatment N~7000-9000 Top 5-10% of the economic pyramid? The Indian Situation Timely referral N = ? Detection N = ? Total CHD population at birth N = 80,000-100,000

Specific Challenges in LMICs Undernutrition Pneumonia Pulmonary hypertension

Photograph obtained and reproduced with permission from family 1999 Baby A: Large VSD and ASD Birth Weight of 3.07 Kg; 2.5 Kg at 6 months! Photograph obtained and reproduced with permission from family

(J Pediatr 2002;140:736-41)

(J Pediatr 2002;140:736-41)

Weight Z Scores > -3: 73; <-3: 125; < -4: 68; < -5: 27 Infant VSDs (Year 2010-2012) – 198 (19.3% of all infant heart operations) Weight Z Scores > -3: 73; <-3: 125; < -4: 68; < -5: 27

3 months after discharge 

90 from original cohort; follow up of 52.3 􏰀± 15.5 months Nutritional Recovery 90 from original cohort; follow up of 52.3 􏰀± 15.5 months J Pediatr 2006;149:205-9;

J Pediatr 2006;149:205-9;

VSD in LMICs: Challenge #2

When does one intervene? What outcomes can be expected? VSD with Severe Respiratory Infections Requiring Mechanical Ventilation Questions: Band vs. Repair? When does one intervene? What outcomes can be expected?

VSD with Severe Respiratory Infections Bhatt M, Roth S, Kumar RK, Gauvreau K, Chengode S, Nair, SG, Shivaprakasha K, Rao SG, Management of infants with large, unrepaired left-to-right shunts and respiratory infection requiring mechanical ventilation, Journal of Thoracic and Cardiovascular Surgery 2004;127:1466-1473.

Institutional criteria for VSD closure in ventilated infants No fever for 48 hours or longer Decreasing leukocyte counts, if initially increased, and increasing platelet counts, if initially reduced At least partial radiologic clearance of lung infiltrates Improved arterial blood gases (PaO2 >􏰁60 mm Hg and PaCO2 <􏰂50 mm Hg on a fraction of inspired oxygen of 􏰂0.4 using pressure-controlled ventilation with maximum peak inspiratory pressures of 30-35 mm Hg and tidal volumes of 10 mL/kg) Unequivocal documentation of predominant left-to-right systolic shunting on color Doppler echocardiography Documentation of negative bacterial cultures and normalization of gas exchange, although desirable, were not mandatory.

VSD with Severe Respiratory Infections Bhatt M, Roth S, Kumar RK, Gauvreau K, Chengode S, Nair, SG, Shivaprakasha K, Rao SG, Management of infants with large, unrepaired left-to-right shunts and respiratory infection requiring mechanical ventilation, Journal of Thoracic and Cardiovascular Surgery 2004;127:1466-1473.

VSD with severe respiratory infections Correction is feasible and should be pursued aggressively Potential to save lives Significant morbidity Postoperative sepsis Prolonged ICU and hospital stay

Specific Challenges in LMICs Undernutrition Pneumonia Pulmonary hypertension

VSD with PAH Generally operable if there is evidence of a significant shunt in the basal state irrespective of PA pressure Age is an important variable and some benefit of doubt must be given to younger patients. However, when in significant doubt, do not operate

Clinical Spectrum of PAH in CHD Operable Clear evidence of a large L-R shunt Typically younger patients Borderline situation: PVR elevated ; operability uncertain. Inoperable: Eisenmenger physiology Shunt reversal Typically older patients

Clearly Operable: Cath not required LV RV LA LV RA RV Clearly Operable: Cath not required

26 year old Blue Single loud S2

Clearly Inoperable: Cath not required RV LV RA LA Clearly Inoperable: Cath not required

Clinical spectrum of post-tricuspid shunts with PAH Operable Failure to thrive,  precordial activity, mid diastolic murmur at apex, Cardiac enlargement, pulmonary blood flow Q in lateral leads on ECG, good LV forces LA/LV enlargement, exclusively L-R flows across the defect Clear clinical /noninvasive evidence of a large left – right shunt Borderline clinical non-invasive data: uncertain operability Cyanosis, quiet precordium, no MDM Normal heart size, peripheral pruning No Q in lateral leads, predominent RV forces No LA LV enlargement, significant R-L flows across the defect Clear evidence of shunt reversal resulting from high PVR. Inoperable

Opportunity to refine data further using the IQIC database Conclusions Large VSDs pose significant challenges in resource limited environments Contextually relevant data available from LMICs to formulate simple guidelines Opportunity to refine data further using the IQIC database

Acknowledgements Patients and their families Former and current members of the AIMS pediatric heart program AIMS administration Children’s Heartlink Steve Roth Children’s Boston Kathy and IQIC