Sindrome di Eisenmenger
Table of Contents
Definizione
La sindrome di Eisenmenger: comprende tutte le cardiopatie congenite (comunicazione interventricolare, comunicazione interatriale, dotto arterioso, isolato o associato ad artifizi congeniti), complicate da ipertensione polmonare e da inversione dello shunt che compare nel periodo perinatale.
La malattia o complesso di Eisenmenger: comunicazione interventricolare alta, con destroposizione dell’aorta, complicata da ipertensione polmonare con inversione dello shunt.
Reazione di Eisenmenger : comparsa tardiva, dopo la pubertà, di una ipertensione polmonare con inversione dello shunt: questa reazione rara, si osserva soprattutto nella comunicazione interatriale, raramente nella comunicazione interventricolare.
Eziologia
Comunicazione interventricolare in un terzo dei casi, più raramente comunicazione interatriale o dotto arterioso. Altre malformazioni possono essere associate. La sindrome di Eisenmenger è più frequente nella donna. La patogenesi dell’ipertensione polmonare è sconosciuta. Una reazione vascolare all’aumento del flusso polmonare scatena un processo di ostruzione progressiva delle arteriole polmonari, durante la vita intrauterina, nel periodo peri-o- neonatale. Questo processo finisce per invertire lo shunt e rende la cardiopatia cianogena.
Sintomi
Clinici
- Cianosi, talvolta più marcata agli arti inferiori, se esiste un dotto arterioso;
- Dispnea
- Emottisi
- Dolori precordiali
- Ippocratismo digitale
- Sincope o morte improvvisa
- Mancanza d’aria (soprattutto in concomitanza di un esercizio fisico)
- Affaticamento
- Letargia
- Vertigini
- Cianosi
- Dolori toracici
- Palpitazioni
- Aritmie atriali e ventricolari
Segni stetoacustici
- Soffio da eiezione breve,” a diamante”, preceduto da un click protosistolico sul focolaio polmonare
- Talvolta soffio diastolico dolce nell’insufficienza polmonare (soffio di Graham Steel)
Esami
Radiologia: arterie polmonari molto grosse che contrastano con l’ iperdiafania della periferia del polmone, dove la trama vascolare è mal visibile. L’angiografia rivela la natura e la topografia delle malformazioni.
Elettrocardiogramma: ipertrofia ventricolare destra, con deviazione assiale sinistra se esiste una comunicazione interatriale di tipo ostium secundum.
Laboratorio: il grado di poliglobulina e l’aumento dell’ematocrito sono indici di gravità di questa cardiopatia.
Cateterismo cardiaco: la pressione e la resistenza vascolare polmonare sono aumentate, mentre la pressione capillare polmonare è normale.
Diagnosi
La sindrome di Eisenmenger deve essere distinta dall’ ipertensione polmonare primitiva, nella quale non vi è il dato anamnestico di soffio nell’infanzia. Bisogna anche distinguere l’ipertensione polmonare secondaria dal cuore polmonare cronico dove il quadro clinico è dominato dalla pneumopatia causale. La stenosi mitralica molto serrata, eccezionalmente il mixoma dell’atrio sinistro e la mediastinite sclerosante con ostruzione delle vene polmonari, possono simulare la sindrome di Eisenmenger.
Prognosi
L’ipertensione polmonare progredisce lentamente e la speranza di vita supera raramente i 40-50 anni. Le complicazioni tromboemboliche e la morte improvvisa sono frequenti.
Terapia
Sintomatica. gli anticoagulanti e i salassi ripetuti non influenzano l’evoluzione. Gli interventi correttivi devono essere effettuati precocemente per prevenire la sindrome di Eisenmenger. Evitare le condizioni che possono esacerbare la malattia (p. es., gravidanza, esercizio isometrico, alte quote, fumo di sigaretta).
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