Ph in achd

Massimo Chessa

Department of Pediatric Cardiology

&

Adult with Congenital Heart Disease

IRCCS- Policlinico San Donato

San Donato Milanese – Milano

massimo.chessa@grupposandonato.it

LE CARDIOPATIE CONGENITE

Cosa sapere in relazione

all’ipertensione polmonare

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Scriveva William Osler (Medico

Canadese 1849-1919) definito a

ragione il padre della medicina

moderna: “... Le cardiopatie congenite

hanno un interesse molto limitato. La maggior

parte si presenta in età neonatale e sopravvive

solo poche settimane o mesi. I casi che

raggiungono un’età adulta sono molto rari…”

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Extraordinary Management Advances Improved Survival

Rate

Year of birth Complex

CHD

Moderate

CHD

Simple

CHD

1940-1959 10% 55% 90%

1960-1979 50% 65% 95%

1980-1989 80% 90% 95%

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Advances

Diagnosis Therapy

Survival

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

The first cause of such increasing

number of patients is

SURGERY

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

More than 75.000 ACHD in Italy

1200-1600 adolescents enter adulthood every year and

require life-long care

More than 85% of infants are expected to reach adulthood

Chessa M, Cullen S, Deanfield J The care of adult patients with congenital heart defects: a new

challenge. Ital Heart J. 2004 Mar;5(3):178-82.

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

It is important to outline that

most pts with CHD who have

had their lives transformed by

surgical intervention, had

Reparative

and not

Corrective Surgery

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Surgery is corrective, if….

…..ventricular function is normal

…..life expetancy is normal!

…..there is no need for therapeutic

measures during f-up

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Corrective Surgery…….

Atrial Septal defect

Ventricular Septal Defect

Patent Ductus Arteriosus

…..if treated during

childhood!!!

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Residuae Sequelae

Mechanical Electrophysiological

Functional

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

CHD-PAH: anatomical-pathophysiological classification

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Clinical classification of congenital

systemic-to-pulmonary shunts associated with PAH

2) PAH associated to systemic-to-pulmonary shunts:

Moderate to large systemic-to-pulmonary shunts (largely

prevalent) (O2 sat >90%).

3) PAH con small defects: similar to idiopathic PAH.

4) Pulmonary arterial hypertension after corrective

cardiac surgery.

1) Eisenmenger syndrome: Large defects and reversed

(pulmonary-to-systemic) or bidirectional shunts (eritrocytosis,

cyanosis: O2 sat <90%).

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Eritrocytosis and ES

Right-to-left shunt

Hypoxemia

Eritrocytosis

Hyperviscosity

Symptoms

Complications

VSD with r-l shunt

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Courtesy of Dr D’Alto M

PSD

GUCH Unit

Ready for phlebotomy?

Avoid routine phlebotomy in ES patients!

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Courtesy of Dr D’Alto M

Phlebotomy in ES patients

“Therapeutic phlebotomy, however, is a ‘two-edged sword’ since

erythropoietin may stimulate the bone marrow to produce

more red cells”.

deplete iron stores

repetitive phlebotomies

iron-deficient red blood cells

reduced O2-carrying and deformability of RBC (microcytes) increased risk of stroke

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Courtesy of Dr D’Alto M

ESC/ ERS 2009

ESC 2010

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Anticoagulation in ES patients?

2009

J Am Coll Cardiol 2009;53:733–40

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

“ Death in ES is difficult to predict.

The following factors suggest it may be imminent

(within next 1-2 y):

1. Pregnancy

2. Cerebral vascular accident

3. Syncope on effort

4. RV failure

5. Fresh haemoptysis at least 100 ml

6. Increasing hypoxia at rest with decreasing effort

tolerance

7. Atrial arrhythmias

8. Ventricular runs (>3 ectopics)

9. Operation requiring general anesthesia

10. Need for pacing

11. Dangerous lifestyle habits (alcohol, disco,

hot bats, sauna, drug abuse)”.

Cause of death in ES patients

Somerville J. Int J Cardiol 1998;63:1-8

Courtesy of Dr D’Alto M

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

It is mandatory a correct organization to

follow this kind of patients

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

National Centre for 5 to 10 milion

people Regional Centre

with some

expertise and

facilities

Primary

Caregivers

Community

Cardiologist

Thank you for your attention

Massimo Chessa massimo.chessa@grupposandonato.it