ph in achd
DESCRIPTION
TRANSCRIPT
Massimo Chessa
Department of Pediatric Cardiology
&
Adult with Congenital Heart Disease
IRCCS- Policlinico San Donato
San Donato Milanese – Milano
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 [email protected]