guch patients, intensive care and registries

GUCH patients, intensive care and registries

G. Agnoletti Turin, Italy

CITTA’ DELLA SALUTE Pediatric Cardiology [email protected]

•  CHD frequency •  CHD survival •  Size of the problem •  Registries (CHD / GUCH) •  Who GUCH patients are? •  Why and when ICU?

CHD frequency •  PAN study: 10 / 1000 (all included)

REGISTER ANGEBORENE HERZFEHLER

No impact of prenatal diagnosis on CHD frequency

children

CHD survival


An increasing population

Marelli, Circulation 2007

Size of the problem

•  Estimated GUCH population > 1 Ml •  250.000 complex diseases (ICU,

interventional, surgical treatment) •  GUCH centers every 17Ml (UK) to 1Ml

(Holland) •  GUCH in a registry : 15.000

Registries for CHD- GUCH ?

•  UK •  Holland •  Spain •  Germany •  Italy DK • Wide experience in Canada (CACH), US….

Dear Gabriella: In Spain there’s not yet a registry on GUCH.... until recently, there were only 4-5 big centers with experience treating these patients.... lately, as you know, GUCH is becoming "fancy" among adult cardiologists, and also the adult interventionalists, bored of stenting the coronary arteries, are increasingly interested in closing ASD, stenting aortas, etc....so an increasing number of centers are dealing with these patients, and unfortunately, they don't always have the needed experience. There is not yet official regulation in this issue, but it will probably come soon ....

un abrazo fuerte

Dear Gabriella, In Germany we do not have a registry on GUCH patients. Within the competence network for congenital heart disease we have a nationwide registry for patients suffering from CHD. This registry comprises so far more than 25000 patients of different age groups. The number of centers dealing with GUCH is appr. 10. Two big centers (Berlin and Munich (both of which > 1000 pts/ year in the outdoor department), and the other ones significantly smaller)

Hope it helps.

CONCOR registry born in 2002 : > 10.000 patients

My French experience

•  The project existed •  The French Society of

Cardiology was willing to provide funds and statisticians

•  Extra work was a problem

My Italian experience

•  Italy is an ensemble of very different regions

•  Registries for congenital anomalies exist but doctors are OBLIGED to use registries in order to receive funds etc…

•  The only European registry is Dutch because Holland is a small country

AEPC cardiac coding

25 + 18 pages (appendix)

Who GUCH patients are and which are their problems ?

Verheugt Circulation 2008

25% complex diseases

Categories of patients •  CHD - native •  CHD - palliated •  CHD after reparative surgery •  CHD after reparative surgery - residual defects •  Cardiac transplantation, heart + lung

transplantation •  CHD and acquired HD

Cardiac problems of the GUCH patient

• Arrhythmias and conduction defects

• Ventricular dysfunction (pressure / volume overload)

• Valvular lesions

• Pulmonary hypertension

• Systemic hypertension

• Aortic disease

• Coronary arteries disease

• Residual shunts (> PBF)

Heart, Lung, Brain, Liver, Vessels

Non cardiac problems of the GUCH patient

• Restrictive-obstructive lung disease (plastic bronchitis)

• Secondary erythrocytosis (cyanosis)

• Coagulation anomalies

• Cholelitiasis -Nephrolithiasis

• Developmental abnormalities

• Central nervous system abnormalities

• Hearing / visual loss

• PLE

Size of the problem CONCOR

•  All hospital admissions 2001-2006 •  28.990/year: 2906 GUCH •  In total 8916 GUCH admissions (61%

cardiovascular indications) •  Most common indication: arrhythmias

(31%) •  50% of interventions: cardiovascular •  Most non cardiovascular admission:

obstetric Verheugt CL Heart 2010

Gender and complications

Verheugt Circulation 2010

Pregnancy

Troubles

Pregnancy and Heart Disease Registry wwww.escardio.org

Pregnancy and Heart Disease

Heart disease: the most frequent cause of death in pregnant women

Saving mothers' lives. CEMACH 2007 Maternal age, congenital…

RISK INDEX DELIVERY IN ICU

•  Cyanosis (SO2 < 90%) or NYHA III- IV (before pregnancy) •  Obstruction to left ventricular outflow •  Dysfunction of the systemic ventricle (EF < 40%) •  Previous cardiac event (pulmonary edema, arrhythmia, cardiac arrest, TIA)

Siu SC et al. Circulation 2001; 104: 515

cTGA, cardiac failure during pregnancy (regressed)

Age and arrhythmias

Van der Velde Eur J Epidemiol 2005

First cause of hospital admission

Mustard, RV (systemic) dysfunction during atrial flutter

Ventricular dysfunction of the GUCH patient

•  Dysfunction of the systemic left ventricle

•  Dysfunction of the systemic right ventricle

•  Dysfunction of the right, subpulmonary ventricle

•  Dysfunction of the single ventricle (R or L or undetermined)

• Role of valve regurgitation

ToF, but too late…

Mortality CONCOR: 6933 pts

• 197 deaths (24/y) •  median age: 48.8 y • 77% cardiovascular

Verheugt Eur Heart J 2010

Mortality 45% CHF or sudden death

Verheugt Eur Heart J 2010

ToF, RV dysfunction, severe TR, CHF

Single ventricle correction

arrhythmias, embolism, cyanosis, dysfunction of the single ventricle, PLE, plastic bronchitis...

Single ventricle

• PBF dependent on systemic venous flow

• IPPV > intrathoracic pressure which < venous return with consequent < PBF and < CO

• >PaCO2 , <pH, <PaO2

• < temperature, pain

• >Peak inspiratory pressure

Pulmonary hypertension

Van der Velde Eur J Epidemiol 2005

Pulmonary problems: Eisenmenger: Heart, Brain, Rein, Coagulation

HEART •  Coronary compression by dilated pulmonary trunk •  Pulmonary embolism

BRAIN •  Stroke •  Cerebral abscess

KIDNEY •  Diminished renal flow •  > urea •  Nephrotic s.

COAGULATION •  Abnormal von Willebrand •  Abnormal endothelium •  Abnormal synthesis-degradation RBC •  Abnormal platelets

Coronary arteries disease

Myocardial ischemia







• Aortic diseases



All CHD with shunt

Non cardiac problems of the GUCH patient

• Restrictive-obstructive lung disease (plastic bronchitis)

• Secondary erythrocytosis (cyanosis)

• Coagulation anomalies

• Cholelitiasis -Nephrolithiasis

• Developmental abnormalities

• Central nervous system abnormalities

• Hearing / visual loss

• PLE

Pulmonary problems

• PROBLEMI RESIDUI

• Increased PBF, increased LAP or obstruction of pulmonary veins flow: interstitial fluid pulmonary compliance Increased respiratory work

• IL PAZIENTE CON Chronic hypoxemia: Increased minute ventilation with = PaCO2 Normal hyperventilation with PaCO2 NO hyperventilation with PaO2

Pulmonary problems

• Scoliosis (19% of patients)

• Phrenic nerve lesions: unilateral diaphragmatic paralysis

Pulmonary problems Plastic bronchitis

Bronchial cast

Hematological disorders • Chronic hypoxemia: > EPO > HTc >hyperviscosity (more important if < Fe++):

Migraine, Vertigo, Blurred vision, Fatigue, Weakness, Paresthesia

Coagulation disorders • Platelets: anomalies of number and function

Single ventricle palliation or correction

cyanosis, embolism, hemoptysis …

The “team”

radiologist

cardiologist

cardiac surgeon

intesivist

endocrinologist

cardiologist

surgeon

nutritionist

infectious disease

gynecologist internist

nephrologist

neurologist

orthopaedist

obstetrician

psychologist

psychiatrist

pulmonologist

sexologist

The patient

geneticist

Open questions

• Were are they ?

• Who takes care of them ?

THE PHANTOM PATIENTS

My mathematics GUCH IN ICU

Germany France Italy Canada Holland

population 82 Ml 60Ml 60Ml 32 Ml 16 Ml

est.GUCH. population

123.000 90.000 90.000 50.000 25000

GUCH in a registry

0 0 0 12000 24%

10000 40%

My mathematics

Germany France Italy UK Spain Holland

population 82 Ml 60Ml 60Ml 51Ml 40Ml 16 Ml

GUCH centers

2-10 1-5 2-5 3 4-5 13

ratio 1/8.2Ml 1/12Ml 1/12Ml 1/17Ml 1/10Ml 1/1.2Ml

Mortality due to acquired-congenital HD

Saving mothers' lives. CEMACH 2007

Congenital From 4,6/1.000.000 in 1996 to 1.9/1.000.000 in 2005

Acquired: From 3,8/1.000.000 in 1990 to 20.8/1.000.000 in 2005

The RV: watch PR

Pulmonary regurgitation depends on:

•  Degree of valvular incompetence

•  Balance between RV diastolic afterload and RV compliance (patients with PH need a valve)

• Duration of diastole

The consequences of pulmonary regurgitation are:

RV dilatation >Increase in end-diastolic RV pressure > Increase of RA pressure >Development of TR > RV dysfunction

Native CoAo: severe systemic hypertension

Anesthesiological risk…

guch patients, intensive care and registries

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