“Resourcelessness of our patients makes them stoic and trusting and that increases our responsibility several folds.” Prof (Dr) SS Kothari [1].
Introduction
Aims/objectives
Methodology: conceptualization of draft
Disability assessment
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Definition of a person with disability (RPWD Act 2016): “a person with long term physical, mental, intellectual or sensory impairment hindering the person’s full, equal and effective participation in society [18].”
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Definition of cardiac disability: “a reduction of productivity to 40% less than expected, in accordance with age and skill due to reduced residual working capacity or need for frequent rest due to chest discomfort, syncope, dyspnea, fatigue, palpitation and cyanosis, leading to absence from work, frequent admission to the hospitals to manage the symptoms of hypoxia, heart failure (HF) and/or arrhythmia [13, 18, 41‐44].”
Step by step process of disability certification of the patients having heart diseases
Broad overview
Heart disease category groups (HDC Gr) | Details of categories of cardiac diseases as diagnosed by cardiologist | Diseases category entry score | ||
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Severity of hemodynamic abnormalities | Necessity of intervention | Expected outcome | ||
HDC Gr Aa | Not significant | No | Good | + 5 |
HDC Gr Bb | Significant | May need eventually | Variable | + 10 |
HDC Gr Cc | Significant | Yes (if suitable for intervention) | Variable | + 20 |
+ 0 | No symptoms (NYHA I) (1) can undertake age-appropriate exercise for at least 30 min at a time; (2) is able to complete any physically active task at home or outside. |
+ 10 | Symptomatic (NYHAII) (1) mild dyspnea, fatigue, and chest pain only occasionally in physically demanding activities—walking to a larger working place without stopping to rest; or performing physically active tasks (e.g., climbing a flight of stairs) or heavier household activities (e.g., sweeping floor or mowing the lawn); (2) is able to perform most work-related tasks, other than tasks involving heavy manual labor (e.g., digging, carrying or moving heavy objects, concreting, bricklaying, laying pavers). (3) acyanotic. |
+ 20 | Symptomatic (NYHA II–III) (1) experiences symptoms, e.g., shortness of breath, fatigue, cardiac pain) when performing day to day activities around the home and community and, due to these symptoms; (2) is unable to walk far outside the home or has difficulty performing day to day household activities (e.g., changing the sheets on a bed or sweeping paths); (3) is able to do tasks like use public transport and walk around in market and can perform work sedentary or stationary nature. (4) acyanotic/ no need for home oxygen therapy |
+ 30 | Symptomatic (NYHA III–IV) (1) The person usually experiences symptoms (e.g., shortness of breath, fatigue, cardiac pain): when performing light physical activities, the person requires assistance to perform light day to day household activities; (2) feels difficulty in sustaining a clerical, sedentary, or stationary for a continuous shift of at least 3 h; ± systemic desaturation SPO2 < 95%. Needing home oxygen therapy at exertion (arterial pO2 ≤ 55 mmHg or SPO2 O2 ≤ 88%) |
+ 40 and above | Symptomatic (NYHA IV). (1) usually experiences symptoms (e.g., shortness of breath, fatigue, cardiac pain) even at rest unable to move around inside the home without the assistance, ± SPO2 < 95%; (2) needs home oxygen therapy at rest (arterial pO2 ≤ 55 mmHg or SPO2 O2 ≤ 88%) |
CDS-subjective criteria for below 14 years of age (maximum score = 40) | |
+ 0 | Asymptomatic (Ross CHF scale grade 1), SPO2 > 95% |
+ 10 | Symptomatic (Ross CHF scale grade 2). Tachypnea but no gross alteration in feeding time/no diaphoresis; SPO2 > 95%; |
+ 20 | Symptomatic (Ross CHF scale grades 2–3). Tachypnea with diaphoresis with altered feeding time in infants/weight gain affected, dyspnea on exertion in older children; 2. SPO2 > 95% |
+ 30 | Symptomatic (Ross CHF scale grade 3). Marked tachypnea and diaphoresis with feeding, prolonged feeding times and growth failure in infants/marked dyspnea on exertion in children; and/or SPO2 < 95% in infants and children |
+ 40 | Symptomatic (Ross CHF scale grade 4). Tachypnea, retractions, grunting, or diaphoresis at rest in infants/marked symptoms in older children at rest; and/or history of cyanotic spell in infants or squatting in older children, ± SPO2 at room air < 95%; further fall in SPO2 on walking (older children) |
+ 10 | Basal ECG or Holter monitoring (± abnormal imaging echo/CT/MRI/PET/cardiac cath) suggestive of significant ischemia, strain, severe ventricular hypertrophy or rhythm disorders, channelopathies, needing intervention; ECHO/CT/MRI (± ECG changes) suggestive of HD (structural, coronary or vascular or pericardial or tumor) associated with serious hemodynamic effects due to anatomical/functional abnormalities; pre/post-intervention HD presenting with functional abnormalities(+ regional wall motion abnormality, LV function < 40%, high PA pressure > mean 40 mmHg/PVRI > 6 woods unit, RV Dysfunction by existing criteria-TAPSE less than expected age-appropriate value; Fr. area shortening = < 30%), ± significant residual lesions or systemic hypertension |
+ 10 | 6-min walk test < 400 m (≃abnormal exercise test, MET< 7, V2Max < 14 ml/kg/min/) ± abnormal stress test (TMT/echocardiogram/thallium test) in presence of HD with significant hemodynamic challenges (exercise, stress test must not be done in critical diseases) |
+ 10 | For evidence of clinical HF(systemic congestion-raised JVP/hepatomegaly, pedal edema or pulmonary congestion/crepts/pulmonary edema, basal tachycardia, bradycardia, or chronotropic incompetence* (abnormal chronotropic index (< 0.8 and > 1.3) - peak HR/resting HR)/(220 - age/resting HR) resulting into radiological HF (cardiomegaly, pulmonary edema, PAH/PVH); biochemical markers of HF (NT-proBNP > 1700 pg/mL or BNP > 140 pg/mL) or systemic hypoxia = Hct > 55% in presence of cyanosis |
+ 10 | Hospital admission records—documenting palliative procedure/cardiac syncope, E/O tachy- or bradyarrhythmia, chronotropic incompetence; presence of PPI, ICD/intervention/decongestive therapy/ionotropic support/requiring non-invasive ventilation/pulmonary vasodilators or therapy for systemic hypertension |
Disability status-Global Disability Scale (% disability) | CDS-HDC group entry (maximum score = 20) Table-1 | CDS-subjective criteria (max score = 40) Table-2 | CDS-objective criteria criteria (max score = 40) Table-3 | CCDS-maximum score = 100 | Disability Certificate (DC) | Expected physical activity |
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No or mild (< 40%) | D0/D1 (DS ≤ 40) | No or some restriction | ||||
Moderate (40–70%) | D1 (DS ≥ 40–70) | Some restriction | ||||
Severe (> 70%) | D1a/D2 (> 70) | Symptom restricted physical activity |
A1. HD-unoperated | |
• Patent foramen ovale (PFO)/small fenestrations at fossa ovalis with or without small aneurysm (< 10 mm) with no chamber dilatation and normal pulmonary artery (PA) pressure, no history of (h/o) cryptogenic syncope • Bilateral superior vena cava (SVC), left SVC to coronary sinus (LSVC-CS) with or without innominate vein but with intact coronary sinus roof • Levo-atrial cardinal vein with normal left atrial (LA) pressure • Isolated inferior vena cava (IVC) interruption with azygos/hemi-azygos continuation | |
• Small fossa ovalis atrial septal Defect (ASD) < 6 mm without chamber dilation or pulmonary hypertension • Partial anomalous pulmonary venous drainage (PAPVC)of single pulmonary vein: without significant chamber enlargement/pulmonary arterial hypertension (PAH)/pulmonary venous hypertension (PVH) • Ebstein’s anomaly grade I/II with mild or low moderate tricuspid regurgitation (TR) with mild chamber enlargement and no atrial shunt • Cleft mitral valve (MV) with no regurgitation • Congenital MV defect without significant gradient/regurgitation/PVH or PAH • Bicuspid aortic valve (AV): with no more than mild regurgitation and mean pressure gradient no more than 20 mmHg, no aortic root dilatation • Valvar pulmonary stenosis (PS) peak gradient < 40 mmHg with no right ventricle (RV) pressure overload • Ventricular septal defects (exception-doubly committed VSD): small causing no pulmonary arterial hypertension/chamber enlargement or aortic valve (AV) prolapsed or aortic regurgitation (AR) • Patent ductus arteriosus (PDA): tiny, not audible even to expert’s ears or incapable of producing complete continuous signal on continuous wave (CW) Doppler and not associated with additional shunt lesion or bicuspid aortic valve or coarctation of aorta with normal echocardiographic evaluation of heart • Flow acceleration at branch pulmonary arteries/ isthmus of aorta (gradient < 18 mmHg) without diastolic spill and RV/left ventricle (LV) volume-pressure (v/p) overload • Mild narrowing of isthmus, or flow acceleration-gradient < 20 mmHg, no diastolic spill, no upper limb hypertension, stationary gradient over 6 months of follow-up | |
A2. HD-underwent corrective surgery/intervention | |
• Acyanotic heart diseases underwent appropriate interventions and have no ventricular dysfunction, dilatation, hypertrophy, significant valvular regurgitation, obstruction, rhythm disturbance, no or mild pulmonary arterial or venous hypertension, systemic hypertension • Few cases of post-op cyanotic congenital heart disease (CHD) like tetralogy of Fallot (TOF) without trans-annular patch (TAP): who do not have residual lesions or ventricular dilatation, hypertrophy, dysfunction, pulmonary hypertension, or arrhythmia • Post pulmonary valvotomy: peak gradient no more than 30 mmHg/no significant RA/RV dilatation or RV dysfunction was noticed on follow-up • MV repair for cleft/prolapse: with no more than mild MR. Normalization of LV-M mode indices on follow-up • Total/partial anomalous pulmonary venous drainage: repair without residual PAH/PVH/arrhythmia • Post-op anomalous origin of left coronary artery from pulmonary artery (ALCAPA), if there no ventricular dysfunction or mitral regurgitation or ECG changes on rest or on exercise | |
A3. HD of hemodynamic significance which improved by palliative intervention | |
• Viral myocarditis or dilated cardiomyopathy due to causes like hypocalcaemia: recovered fully with normal ventricular function/ normal chamber size and no more than mild MR regurgitation • Cardiac tumor like rhabdomyoma regressed completely • Mild hypertrophic cardiomyopathy (HCM): the patients having no significant intra cardiac gradients. No history of syncope, arrhythmia, or systolic anterior motion of mitral leaflet or MR and no progression over the period of time • Muscular VSD closed completely or became hemodynamically insignificant post PA band ± PA de-banding with normal RV pressure • Lesions (ASD/VSD/PDA/AS/PS/MR/TR) is static over the repeated cardiac evaluation and have no ventricular dilatation or PAH • Rheumatic heart disease (RHD): 1. regurgitant lesion reverted to no or mild regurgitation and patient is on continuous secondary prophylaxis • Kawasaki disease (KD): has coronary dilatation which has reverted back to normal on follow-up period. Normal ECG (electrocardiogram) and TMT (treadmill test) • Pulmonary stenosis valvar, supravalvar, sub-valvar: gradient < 30 mmHg peak; not increasing over the period of time • Aortic stenosis (AS)—s/o valvar, sub-valvar or supravalvar: gradient < 20 mmHg mean; static lesions, over the period of the time and no ventricular hypertrophy or dysfunction • Mitral stenosis (MS)/supramitral ring (SMR): mean gradient < 5 mmHg and remains static, no evidence of PAH or pulmonary edema, right heart dilatation, patient is on regular secondary prophylaxis • Heterotaxy syndrome without cardiac malformations or arrhythmia (isomerism of left atrial appendage may not have significant heart defect) | |
A4. Systemic arterial hypertension /pulmonary arterial hypertension or pulmonary venous hypertension ± hemodynamically insignificant pre- or post-operative structural heart disease | |
• Pulmonary hypertension: primary PAH, attitude PAH, post-op residual PAH (mean PAP < 30 mmHg), no RVH or RV dilatation, normal IVC • Borderline or mild systemic hypertension-well controlled on lifestyle management or one or combination of drugs | |
A 5. Dominant arrhythmia ± hemodynamically insignificant pre or post-operative structural heart disease | |
• No recurrence after initial few episodes needing medication or cured by radio-frequency ablation • Evidence of (E/O) WPW (Wolff-Parkinson-White syndrome) syndrome on electrocardiogram (ECG) but no episodes of SVT (supraventricular arrhythmia) |
B1. HD-unoperated | |
• Pre-tricuspid shunt-fossa ovalis ASD; SV (sinus venosus) ASD; primum ASD (± small VSD, mild MR); CS (coronary sinus) ASD with chamber dilation mild or moderate pulmonary hypertension/no h/o palpitation, no significant MR or TR or pulmonary venous stenosis, no associated heterotaxy syndrome. PFO with cryptogenic stroke • Partial anomalous pulmonary venous drainage of one or two veins: with some chamber enlargement but no or mild PAH/PVH • Acyanotic post-tricuspid shunts-ventricular septal defects: doubly committed VSD/perimembranous/muscular/inlet VSD mild to moderate pulmonary arterial hypertension (PAP less than 2/3rd systemic)/with some chamber enlargement mild aortic valve prolapse (doubly committed VSD), with or without trivial aortic regurgitation; patent ductus arteriosus: continuous murmur/capable of producing complete continuous signal on CW Doppler and associated with no significant additional shunt lesion or obstructive or regurgitant bicuspid aortic valve with normal or borderline enlargement of LA/LV and mild to moderate PAH • Coronary AV fistula(AVF), pulmonary AV fistula with mild cyanosis • Ebstein’s anomaly grade I/II with moderate TR with significant chamber enlargement/right to left shunting at atrial level • Corrected transposition (c-TGA), ± mild to moderate PS, no TR, no arrhythmia, no LV regression • Cleft mitral valve/congenital mitral valve defect with moderate gradient / mild to moderate regurgitation but no PVH or PAH • Bicuspid aortic valve: with mild to moderate regurgitation with chamber dilation (LVIDd (Left ventricular internal dimension-diastole) < 3.8 cm/m2; LVIDs (LVID-systole) < 2.6 cm/m2) and/or aortic stenosis with mean PG no more than 40 mmHg and no significant LVH in presence of normal ventricular function • Valvar pulmonary stenosis peak gradient less than 60 mmHg with no significant RV pressure overload • Posterior shelf at isthmus, ± hypoplastic arch, gradient ≤ 20 mmHg, but mild diastolic spill, mild upper limb hypertension, no LVH (left ventricular hypertrophy), normal EF (ejection fraction) • Branch pulmonary arteries stenosis with mild RV hypertension Lung perfusion scan mildly abnormal • Unoperated rheumatic/degenerative valvar diseases with mildly symptomatic patients • Post Kawasaki small aneurysm of coronary artery disease no cardiac dysfunction, regional wall motion abnormality, MR • Asymptomatic constrictive Pericarditis diagnosed on echo/CT (computed tomography imaging)/MRI/cath—mild JVP rise or hepatomegaly, no ascites • Hypertrophic cardiomyopathy with LVOT obstruction < 35 mmHg or not symptomatic in more than routine activity. • Dilated cardiomyopathy LVEF > 40% • Asymptomatic restrictive cardiomyopathy mild or moderately raised PA pressure ▪ Coronary heart disease in adults with no significant symptoms and amenable to intervention. Systemic hypertension controlled with multidrug therapy with or without end organ effects. ▪ Marfan’s syndrome or other familial thoracic aortic aneurysm syndromes (Loeys-Dietz syndrome [due to TGFBR1 and TGFBR2 mutations] and those related to mutations in SMAD3, TGFB2, and TGFB3), if aortic root is dilated but < 40 mm and no significant mitral tricuspid regurgitation (due to nature of disease, it cannot be kept in Table 5). | |
B2. HD-underwent corrective surgery/intervention (Evaluation after 12 months) | |
Post-surgery congenital/valvar/pericardial/coronary heart disease. Evaluation after 12 months’ revealed persistent mildly or moderately increased chamber size and mild to moderate MR/AR or moderate to severe TR/severe PR/mild to moderate residual gradient across the treated valve/mild or moderate PAH/mild pulmonary venous stenosis of > 2 veins; LVEF 40-50% | |
• Pre- or post-tricuspid shunt lesions, coronary AV fistula (post-intervention)-with residual shunt >2 mm, ventricular dilatation (LV Z score >2) mean PAP >25<40 mmHg • TOF/DORV (double outlet right ventricle)/D-TGA (D-transposition of great vessel or ventricular arterial discordance)/C-TGA (corrected-transposition of great vessels or atrio-ventricular or ventricular arterial discordance). Anatomical repair-with mild or no RVOT (RV outflow tract) obstruction or LVOT (LV outflow tract)obstruction/mild or low moderate regurgitation/no ventricular dysfunction, regional wall motion abnormalities/aortic root dilatation (Z score >+ 2 or progressive dilatation), mild or no ECG abnormality; residual shunt lesions (> 2 mm causing ventricular dilatation or Pulmonary hypertension) • Coarctation of aorta: neonatal or infantile coarctation: with mild residual gradient and systemic hypertension, ventricular hypertrophy, or dysfunction; coarctation of late childhood: stenting with no or mild residual lesion and hypertension • Bicuspid aortic valve: post ballooning: mean gradient < 40mmHG, aortic regurgitation. On follow-up, no echocardiographic or functional deterioration or more than mild dilatation or mild LVH was noticed • Post-op anomalous origin of any of coronary artery from pulmonary artery (ALCAPA) underwent corrective procedure and has mild ventricular dysfunction or MR | |
B3. HD of hemodynamic significance which improved by palliative intervention | |
• Systemic to pulmonary artery shunt, Glenn shunt; Fontan/TCPC (total cavo-pulmonary connection) surgery, symptomatic in more than routine activity or having basal SPO2 > 80% • Shunt procedure or RV to PA conduit palliation ± with or without unifocalization of MAPCA (major aorto-pulmonary collateral artery)-as a final procedure for complex CHD with hypoplastic ± nonconfluent ± absent central pulmonary arteries • PA banding as a final procedure for complex CHD or multiple VSDs or VSD with tricuspid or mitral valve straddling or unbalanced AVSD (atrioventricular septal defect) with SPO2 > 85%, without AV regurgitation, cardiac dysfunction • Palliative arterial switch or Senning done for TGA + VSD or DORV + subpulmonary VSD | |
B4. Systemic arterial hypertension/pulmonary arterial hypertension or pulmonary venous hypertension ± hemodynamically insignificant pre or post-operative structural heart disease | |
• Primary Pulmonary hypertension or post-operative residual PAH: PA mean pressure < 40 mmHg. • Systemic hypertension with or without mild end organ defect on multidrug therapy. Normal LVEF. | |
B5. Dominant arrhythmia ± hemodynamically insignificant pre- or post-operative structural heart disease | |
(Controlled but not cured) • Bradycardia not needing pacemaker • Bradycardia with pacemaker • Primary tachyarrhythmia controlled on adequate medical therapy or amenable to RFA, long QTc with no episodes of ventricular tachycardia • Complete heart block with narrow QRS complex escape rhythm and HR > 70/min, without chamber dilatation and cardiac dysfunction • CHB with pacemaker • Long QT syndrome (LQTS), Brugada syndrome (BrS), early repolarization syndrome, short QT syndrome, and possibly idiopathic VF Catecholaminergic polymorphic ventricular tachycardia (CPVT) |
C 1. HD-unoperated | |
• Pre-tricuspid shunt (amenable to intervention)-atrial septal defect/PAPVC (partial anomalous pulmonary venous connection) ± RV dysfunction, or AV valve regurgitation or rhythm dysfunction or moderate to severe pulmonary hypertension • Post-tricuspid shunt. With controlled or uncontrolled CHF-ventricular septal defect, AP window, patent ductus arteriosus, large coronary AV fistula, complete atrioventricular septal defect (AVSD) with balanced/unbalanced ventricles ± AV valve regurgitation ± LVOT or RVOT obstruction • Shunt lesion with SPO2 < 95% saturation on room air (Eisenmenger syndrome) • Obstructive lesions (sub-valvar, valvar, supravalvar-AS/PS) and vascular (pulmonary artery stenosis/coarctation of aorta) defect with pressure overload of respective ventricular chamber, with or without ventricular dysfunction (amenable to cath/surgical intervention) or MS, supravalvar MS with dilatation of left atrium, pulmonary venous congestion, RVH, TR, PAH; TS with RA dilatation, systemic venous congestion • Regurgitant lesions: mitral, aortic, tricuspid regurgitation (needing intervention according to the existing guidelines), pulmonary regurgitation with right heart dilatation • Large coronary AV fistula needing neonatal or infantile intervention • Cyanotic congenital heart disease (± arch anomaly)-Amenable for surgical intervention-tetralogy of Fallot (TOF); DORV + VSD ± RVOTO(RVOT obstruction)/LVOTO(LVOT obstruction); transposition of great arteries ± VSD, ± PDA ± LVOTO; corrected transposition with VSD ± PS with or without systemic severe TR; AVSDPS or PAH; AVSD-unbalanced ventricles; truncus arteriosus (TrA) with or without truncal valve stenosis or regurgitation; TrA with arch interruption; tricuspid Atresia/VSD+/-PS; Single ventricle VSD ± PS; TAPVC ± obstruction; heterotaxy syndrome with hemodynamically significant cardiac malformations; hypoplastic left heart syndrome and all complex congenital heart diseases with SPO2 < 90% ± cyanotic spell ± congestive heart failure • Cyanotic CHD with PAH/PS with small or absent PA’s large MAPCA-unsuitable for surgical or cath intervention-Eisenmenger Syndrome with Cyanotic CHD (increased blood flow-DORV or TGA + VSD without PS, single ventricle/tricuspid atresia without PS, TAPVC, truncus arteriosus) • Complex left-sided obstructive lesions like congenital mitral stenosis • Pulmonary AV fistula-significant systemic desaturation, sometimes involving significant part of lung and show poor outcome after intervention • Complex anatomy (unsuitable anatomy) mitral stenosis/regurgitation with or without ventricular hypoplasia, Shone complex in small children; veno-occlusive disease of pulmonary veins • Systemic very large-cranial AV fistula needing early intervention • Ebstein’s anomaly with severe TR, unguarded TV ± PFO/ASD, severely dilated RA + right to left shunt at PFO; pulmonary atresia with or without VSD; single ventricle PS/PAH • Cardiac tumors ± arrhythmia or diastolic dysfunction • Uhl’s anomaly of RV/RA appendage aneurysm • Rheumatic/degenerative valvar diseases with persistent congestive heart failure • Post Kawasaki significant aneurysmal or stenotic coronary artery disease leading to moderate to severe cardiac dysfunction, regional wall motion abnormality, MR • Premature coronary artery disease in children and adolescent (vasculitis syndromes, metabolic syndrome hypercholesteremia) with persistent LV dysfunction or suboptimal result of revascularization surgery or intervention are expected • Coronary heart disease in adults with significant angina, dyspnea on exertion, or heart failure • Symptomatic constrictive pericarditis more than NYHA class II symptoms • Hypertrophic (obstructive) cardiomyopathy (HCM/HOCM) with LVOT obstruction > 35 mmHg or symptomatic more than routine activity (particularly if there is history of previous cardiac arrest, syncope, ventricular arrhythmias, family history of sudden death, extreme LV hypertrophy, and a blunted blood pressure response to exercise • Dilated cardiomyopathy (DCMP) LVEF < 40%, chamber dilatation, partially controlled or uncontrolled CHF ± MR • Restrictive cardiomyopathy if symptoms are happening in routine activity and there is moderate to severe pulmonary hypertension | |
C2. HD-underwent corrective surgery/intervention (Evaluation after 12 months of intervention) | |
• Post-op patients with hemodynamically significant residual lesions (valvar stenosis or regurgitation), ventricular dysfunction, systemic desaturation (may or may not be amenable to surgery) • Operated cyanotic and acyanotic CHD with non-resolving pulmonary hypertension requiring medical management • Post coarctation/aortic repair or intervention (ballooning, stenting or vascular graft) ± residual gradient with significant systemic hypertension • Post CABG or coronary stenting with no recovery of ventricular dysfunction or presence of ventricular arrhythmia • Post heart or heart and lung transplant | |
C 3. HD of hemodynamic significance which improved by palliative intervention | |
• Systemic to pulmonary artery shunt, Glenn shunt, with significant systemic desaturation • Fontan/TCPC with complications like liver cirrhosis, plastic bronchitis, protein-losing enteropathy (PLE), systemic desaturation (SPO2 < 80%) • Primary pulmonary hypertension with or without palliative procedure like ASD creation or Pott’s shunt • PA banding as a final procedure for complex CHD or multiple VSDs or VSD with tricuspid or mitral valve straddling or unbalanced AVSD, presenting late with symptoms, not amenable to biventricular repair, systemic desaturation (SPO2 < 80%) or persistent CHF • Shunt procedure or RV to PA conduit palliation ± with or without unifocalization of MAPCA (major aortopulmonary collateral artery)-as a final procedure for complex CHD with hypoplastic ± nonconfluent ± absent central pulmonary arteries • Palliative arterial switch or Senning operation done for TGA + VSD or DORV + subpulmonary VSD | |
C4. Systemic arterial hypertension /pulmonary arterial hypertension or pulmonary venous hypertension ± hemodynamically insignificant pre or post-operative structural heart disease | |
• Primary pulmonary hypertension with symptoms or post-op residual PAH (a resting mean pulmonary artery pressure (mPAP) of ≥ 30 mmHg and a normal pulmonary capillary wedge pressure (PCWP) of ≤ 15 mmHg, PVR > 240 dyn × s × cm-5). • PPH with severe RV dysfunction. Severe systemic desaturation in presence of natural or therapeutic shunt. • Systemic hypertension with secondary organ dysfunction, cardiac dysfunction | |
C 5. Dominant arrhythmia ± hemodynamically insignificant pre or post-operative structural heart disease | |
• Primary channelopathies, tachy-/bradyarrhythmias with history of life-threatening arrhythmia or with ICD • Arrhythmogenic right ventricular dysplasia-with or without history of syncope and arrhythmia, severe RV dysfunction • Non-resolving post-op arrhythmia in presence of ventricular dysfunction, hypertrophy, dilatation, pulmonary venous or arterial hypertension • Any CHD/VHD or coronary heart disease with ICD implantation • Any treated or untreated CHD/VHD with rhythm disturbances requiring EP study, RFA which is expected to have sub-optimal outcome • Primary rhythm disturbance with poor outcome of EP procedures • Intractable primary tachyarrhythmia, long QTc with episodes of ventricular tachycardia • Complete heart block |
Summary (step by step process of disability certification of the patients having heart diseases)
Heart diseases (HD) and heart diseases categories (HDC)—groups A, B, and C [2, 3, 5‐7, 9, 13, 22‐54]
Heart diseases category group A—expected CCDS < 40 (Table 1)
Heart disease category group B—expected CCDS 40–70 (Table 1)
Heart diseases category group C—expected CCDS > 70 (Table 1)
Subdivision of heart disease category groups
Cardiac disability score—subjective criteria (Table 2) [13, 18‐20, 40‐47]
Cardiac disability score—objective criteria [12, 13, 41‐49]
Comprehensive cardiac disability score disability scale and disability certificate (Table 4)
Disability certificates (Table 4)
Certificate D0
Certificate D1 (temporary disability certificate)
Certificate D2 (permanent disability certificate)
Prefixing the disease category along with the disability certificate
Case scenario (disability certificate ( D-0,1,2) prefixed with HDC category -A,B,C and subdivisons 1-5) | Description: heart disease category group (HDC Gr); subdivision; comprehensive cardiac disability score (CCDS); expected disability certificate (DC) | Diagnosis |
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1. A 5-year-old child was certified as AIDO | HDC Gr A (no or mild hemodynamic abnormalities with least chances of progression and intervention) Subdivision: 1 (HD-unoperated) Expected CCDS: < 40 (< 40% 0n disability scale) Expected DC: D0 (predicts none or mild disability) | Restrictive perimembranous VSD |
2. A 5-year-old child with certificate as B3D1 | HDC Gr B (significant hemodynamic effect not needing intervention now but, high chances of progression, and intervention in future) Subdivision: 3 (HD with hemodynamic significance but improved by palliative intervention) Expected CCDS: 40–70 (40–70% on disability scale) Expected DC: D1 (it is possible to improve functional capacity significantly by doing an intervention) | Post Glenn shunt SPO2 = 85% |
3. A 5-year-old patient as B4D1 | HDC Gr B (significant hemodynamic effect, not needing intervention now but , high chances of progression and intervention in future) Subdivision: 4 (systemic arterial hypertension/pulmonary arterial hypertension or pulmonary venous hypertension ± hemodynamically insignificant pre or post-operative structural heart disease) Expected CCDS: 40–70 (40–70% on disability scale) Expected DC: D1 (it is possible to improve functional capacity significantly by doing an intervention) | Primary pulmonary hypertension (PA mean 35 mmHg) |
4. A 5-years-old child certified as B1D1 | HDC Gr B (significant hemodynamic effect, high chances of progression and intervention) Subdivision: 1(HD-unoperated) Expected CCDS: 40–70 (40–70% on disability scale) Expected DC: D1 (it is possible to improve functional capacity significantly by doing an intervention) | Large fossa ovalis ASD, no PAH/arrhythmia |
5. A 45-year-old male patient as B3D1 | HD Gr B (significant hemodynamic effect, not needing intervention now but high chances of progression and intervention in future) Subdivision: 3 (HD with hemodynamic significance but improved by palliative intervention) Expected CCDS: 40–70 (40–70% on disability scale) Expected DC: D1 (it is possible to improve functional capacity significantly by doing an intervention) | Post coronary artery stenting doing well. LVEF 40% |
Benefits for the patients grouped under the disability grading system
Discussion and review of literature
Prevalence of heart diseases
Studies | Age group (year) | Setting | Place of study | Total No. | Screening method | No. with CHD | Prevalence per 1000 |
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Gupta et al. 1992 | 6-16 | Community | Jammu | 10,263 | Clinical | 8 | 0.8 |
Vashishtha et al. 1993 | 5-15 | School | Agra | 8449 | Clinical | 44 | 5.2 |
Thakur et al. 1995 | 5-16 | School | Shimla | 15,080 | Clinical | 30 | 2.25 |
Chadha et al. 2001 | < 15 | Community | Delhi | 11,833 | Clinical | 50 | 4.2 |
Misra et al. 2009 | 4-18 | School | Eastern Uttar Pradesh | 118,212 | Clinical Echo for suspected cases only | 42 | 1.3 |
Kumari et al. 2013 | 5-16 | School | Dist. Prakasam, Andhra Pradesh | 4213 | Clinical and echo in all | 39 | 9.2 |
Saxena et al. 2013 | 5-15 | School | Ballabgarh, Haryana | 14,716 | Clinical Clinical and echo | 3577 | 2.37 5.23 |
Bhardwaj et al. 2016 | All age/<18years | Community | Himachal Pradesh | 1882/760, <18years | Clinical; echo in suspected cases | 12 All/9.89<18years | 6.31 (all) 12.95(<18 years) |
Percentage of total deaths (95% UI) | Percentage of total DALYs (95% UI) | |||||
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Both sexes | Men | Women | Both sexes | Men | Women | |
Cardiovascular diseases | 28.1% (26.5–29.1) | 29.2% (27.5–30.3) | 26.7% (23.8–28.3) | 14.1% (12.9–15.3) | 15.8% (14.5–17.1) | 12.2% (10.9–13.4) |
Ischemic heart disease | 17.8% (16.8–18.5) | 19.6% (18.5–20.4) | 15.6% (13.9–16.6) | 8.7% (7.9–9.5) | 10.4% (9.5–11.3) | 6.6% (5.9–7.4) |
Stroke | 7.1% (6.6–7.5) | 6.9% (6.4–7.3) | 7.3% (6.5–7.9) | 3.5% (3.2–3.9) | 3.6% (3.3–4.0) | 3.4% (3.0–3.8) |
Hypertensive heart disease | 1.3% (1.1–1.5) | 1.1% (0.9–1.4) | 1.6% (1.2–1.9) | 0.6% (0.5–0.7) | 0.6% (0.5–0.7) | 0.7% (0.5–0.8) |
Rheumatic heart disease | 1.1% (1.0–1.2) | 0.8% (0.7–0.9) | 1.5% (1.3–1.7) | 0.8% (0.7–0.9) | 0.7% (0.6–0.7) | 1.0% (0.9–1.1) |
Atrial fibrillation and flutter | 0.21% (0.16–0.26) | 0.17% (0.13–0.21) | 0.25% (0.20–0.32) | 0.13% (0.11–0.16) | 0.13% (0.10–0.15) | 0.15% (0.12–0.18) |
Aortic aneurysm | 0.15% (0.14–0.17) | 0.20% (0.18–0.21) | 0.10% (0.09–0.11) | 0.07% (0.07–0.08) | 0.10% (0.09–0.11) | 0.05% (0.04–0.06) |
Other cardiovascular and circulatory diseases | 0.14% (0.09–0.17) | 0.13% (0.07–0.18) | 0.15% (0.08–0.18) | 0.07% (0.05–0.08) | 0.07% (0.05–0.09) | 0.07% (0.05–0.08) |
Cardiomyopathy and myocarditis | 0.12% (0.09–0.13) | 0.12% (0.09–0.15) | 0.11% (0.07–0.13) | 0.11% (0.08–0.12) | 0.11% (0.08–0.13) | 0.10% (0.06–0.12) |
Endocarditis | 0.12% (0.10–0.15) | 0.11% (0.09–0.16) | 0.14% (0.11–0.18) | 0.07% (0.06–0.09) | 0.07% (0.06–0.10) | 0.08% (0.06–0.10) |
Peripheral artery disease | 0.01% (0.07–0.03) | 0.02% (0.01–0.04) | 0.01% (0.01–0.02) | 0.02% (0.01–0.03) | 0.02% (0.01–0.03) | 0.02% (0.01–0.03) |