Fetal MRI can provide additional useful information when evaluating congenital heart disease (CHD) prenatally. It may help characterize complex CHD and detect associated extracardiac abnormalities when ultrasound is inconclusive. Fetal MRI is particularly helpful in the late second and third trimesters as ultrasound can be limited by decreasing amniotic fluid and ossifying ribs. It allows assessment of organs like the lungs, thymus and brain that may be involved with certain CHD or genetic syndromes.
Fetal echocardiography provides several advantages for diagnosing and managing congenital heart disease:
1) It allows for counseling of parents on prognosis and treatment options, including the possibility of termination of pregnancy for major defects or chromosomal anomalies.
2) In some cases, it enables life-saving procedures to be performed on the mother or fetus before or during pregnancy.
3) It facilitates delivery planning at a specialized medical center near pediatric cardiac surgery.
This document summarizes a presentation about the 5D Heart ultrasound solution for fetal echocardiography. It discusses how 5D Heart uses intelligent navigation technology to automatically display nine standard fetal echocardiography views from a STIC volume dataset. It can generate the views consistently regardless of operator skill and works well for different fetal ages, sizes, and positions. The views include labels for main anatomical structures.
This document discusses the diagnosis and management of fetal tachyarrhythmias. It begins by outlining the mechanisms, risks, and history of recognizing and treating fetal supraventricular tachycardia (SVT) and atrial flutter. It then poses 4 questions to guide the safest and most effective management: 1) What is the mechanism?; 2) What is the fetal tolerance?; 3) Who to treat?; and 4) How to treat? It discusses using echocardiography, Doppler, tissue velocity imaging, and magnetocardiography to determine the mechanism. It outlines signs of heart failure and hydrops as indicators of risk. It recommends treating fetuses with hydrops or high risk of hydrops
21 s placid diagnosi e terapia delle bradiaritmie fetaliPiccoloGrandeCuore
This document summarizes the diagnosis and treatment of fetal bradyarrhythmias. It discusses that fetal bradycardias can be caused by blocked atrial bigeminy, sinus bradycardia, fetal distress, congenital heart disease, long QT syndrome, or atrioventricular block. Atrioventricular block occurs in 70% of cases and can be associated with congenital heart disease, isolated and immune-mediated, or functional 2:1 AV block in long QT syndrome. For isolated congenital AV block that is antibody-mediated, treatment with corticosteroids may help but outcomes depend on gestational age and heart rate at diagnosis. Postnatal pacemaker implantation is often needed, especially if risk factors like
This document summarizes a presentation on the fetal diagnosis of complete vascular rings caused by right aortic arch and left ductus arteriosus. It discusses the anatomy, embryology, and patterns seen in this condition. Out of 19 patients studied, 7 were prenatally diagnosed between 26-28 weeks gestation. For the fetal population, all were asymptomatic and 2 required surgery. The postnatal population was more commonly symptomatic, with 9 of 12 requiring surgery. Surgical resection of the left ligamentum was performed in 11 of the 19 patients. The presentation concludes that fetal diagnosis of this condition is easier than postnatal diagnosis, and recommends CT/MRI imaging and long-term follow up.
This document proposes a new international project called "Heaven Can Wait" to address critical congenital heart disease in developing countries. It involves establishing prenatal cardiac screening programs and transferring high-risk fetal-maternal pairs to centers in Europe for delivery and cardiac surgery. This aims to give neonates the best chance for treatment and survival, while also improving the efficacy of humanitarian cardiac organizations by providing them with prenatally detected cases. Though large-scale implementation faces challenges, the project envisions adding a new dimension to fetal and perinatal cardiology to help address the stark contrast in outcomes between developed and developing world contexts.
This document provides guidelines for fetal aortic valvuloplasty procedures. It describes the selection criteria for the procedure, which aims to promote ventricular growth and function in fetuses with evolving hypoplastic left heart syndrome (HLHS). The guidelines specify that the dominant cardiac anomaly must be aortic stenosis, with signs of evolving HLHS and potential for a technically successful procedure and biventricular outcome. The procedure involves accessing the fetal heart through needle puncture under ultrasound guidance and using catheters to dilate the stenotic aortic valve.
04 b marino malattie cardiache congenite e sindromi genetiche PiccoloGrandeCuore
This document discusses congenital heart diseases and genetic syndromes. It provides an overview of epidemiological studies on non-cardiac malformations associated with congenital heart diseases. It also discusses the embryology of the heart. The document outlines new clinical roles for genetics, including reverse medicine using new diagnostic criteria, predictive medicine correlating genes to prognosis, and potential future applications of gene therapy and stem cell therapy. Specific genetic syndromes are examined in depth, including their cardiac defects, surgical outcomes, post-operative complications, and prognosis. These include Down syndrome, del22q11 syndrome, Noonan syndrome, and LEOPARD syndrome. The document emphasizes the importance of predictive medicine in correlating a patient's genetic syndrome to clinical prognosis
1. Ospedale Pediatrico Bambino Gesù, Cardiologia
G. Rinelli
Follow Up
a medio e lungo termine
delle
Cardiopatie Congenite
F.Up delle Cardiopatie Congenite - G.Rinelli -
4. F.Up delle Cardiopatie Congenite - G.Rinelli -
Complesse
Semplici
Gruppo Fisiopatologico
Anatomica
Prognosi
Classificazione
> 2 lesioni
(No DIA e Dotto)
15. Gunnar Erikssen et al. Circulation. 2015;131:337-346
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
16. Gunnar Erikssen et al. Circulation. 2015;131:337-346F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
17. Gunnar Erikssen et al. Circulation. 2015;131:337-346
AVSD atrioventricular septal defect; I/HAA, interrupted or hypoplastic aortic arch; PA, pulmonary atresia; TAC, truncus
arteriosus communis; TAPVD, totally anomalous pulmonary venous drainage; TGA, transposition of the great arteries; TOF,
tetralogy of Fallot; and UVH, univentricular hearts.
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
18. Cumulative postoperative survival until 16 years of age or the
end of follow-up in the combined group of patients with complex
congenital heart defects. Gunnar Erikssen et al. Circulation. 2015;131:337-346
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
19. Gunnar Erikssen et al. Circulation. 2015;131:337-346
Number of deaths related to congenital heart defects (International Classification of
Diseases, 10th Revision codes Q20–Q28) in Norway from 1971 to 2011. The red line
includes patients aged 0 to 15 years at the time of their death, and the blue line
includes patients aged 15+ years at the time of their death
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
20. Kaplan–Meier plots of reoperation-free survival in the combined group of patients with complex
congenital heart defects who had their index operation in 1990 to 1999 vs 2000 to 2011.
Gunnar Erikssen et al. Circulation. 2015;131:337-346F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
21. One-year survival in patients with complex congenital heart defects born in 1975 to 1979, 1980
to 1984, 1985 to 1989, 1990 to 1994, 1995 to 1999, 2000 to 2004, 2005 to 2009, and 2010 to 2011.
Gunnar Erikssen et al. Circulation. 2015;131:337-346
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
22. F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza
1 anno di età 80%
15 anni di età 72%
Knowles RL et al. PLoS One. 2014 Sep 10;9(8):e106806. doi: 10.1371/journal.pone.0106806. eCollection 2014.
Prognosi
33. F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Tetralogia di Fallot
Intervento correttivo (patch transanulare)
Pazienti corretti ma senza valvola polmonare
In adolescenza tutti dovranno impiantare una
valvola polmonare
Chirurgia vs Cateterismo cardiaco
38. Giornate Akragantine di Cardiologia Pediatrica - CAV -
CAV Completo
Down Non Down
Secondo Clinica
Entro 4 Mesi Secondo
Clinica
Prognosi
Buona
Prognosi
Buona
Ipoplasia VS
Prognosi
variabile
Prognosi
Canale Atrio Ventricolare
39. CAV Parziale
In genere timing come un
Difetto Interatriale
Secondo
Clinica
Insufficienza Mitralica
Ipertensione Polmonare
Prognosi Ottima
Prognosi
Canale Atrio Ventricolare
40. CAV Parziale con S. Shone
1 Stadio Coartazione
2 Stadio CAVp
Mitrale ?
Prognosi da molto buona ad infausta a secondo
della ipoplasia della cavità sinistre
Prognosi
Canale Atrio Ventricolare
57. F.Up delle Cardiopatie Congenite - G.Rinelli -
APSI
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346
58. F.Up delle Cardiopatie Congenite - G.Rinelli -
Atresia Polmonare
con
Difetto Interventricolare
Prognosi
59. Tratto d’efflusso ventricolare destro:
- atresia infundibolare
- atresia giunzione infundibolo-polmonare
Difetto interventricolare: v. t. di Fallot con SP
ampio DIV conoventricolare da malallineamento
perimembranoso (continuità fibrosa tricuspido-
aortico-mitralica nel suo margine posteriore)
muscolare (20% dei casi)
Sorgenti di flusso ematico polmonare:
- dotto arterioso: tipo A
- collaterali sistemico-
polmonari maggiori
(MAPCAs): tipi B e C
Arterie polmonari:
- ben rappresentate
- ipoplasiche ± difettive
- discontinue / assenti
Type A
Type B Type C
AP + DIV –Anatomia
60. No Mapcas (Tetralogia di Fallot con Atresia Polmonare)
Shunt
Correzione
(Dopo pochi mesi)
F.Up delle Cardiopatie Congenite - G.Rinelli -
AP + DIV (Tipo TOF)
61. Basi della Chirugia Correttiva
Necessità di convertire il pattern multifocale di
perfusione polmonare in una fonte unica:
concetto di
UNIFOCALIZZAZIONE
F.Up delle Cardiopatie Congenite - G.Rinelli -
AP + DIV + MAPCAS
62. Chirurgia
Diagnosi: Eco
Ricostruzione palliativa tratto efflusso VD
Procedure di unifocalizzazione:
Chiusura DIV (Correzione completa)
Cateterismo
Chirurgia
F.Up delle Cardiopatie Congenite - G.Rinelli -
AP + DIV + MAPCAS
Scelta Procedura
63. Timing Chirurgico
Diagnosi: Eco
1-5 Mesi Cateterismo
Dipende da Sat O2
Paziente Stabile Cianotico
1 ° Chirurgia 5-8 Kg
Paziente Stabile Poco Cianotico
o Iperafflusso Polmonare
4-5 Mesi Cateterismo
Chirurgia
Cataterismo Cardiaco di controllo/interventistico dopo la correzione e durante i vari stadi
Condotto VD- AP + Unifocalizzazione
Chiusura DIV
F.Up delle Cardiopatie Congenite - G.Rinelli -
AP + DIV + MAPCAS
64. January 1994 – September 2014
Number of pts
1994-2000
2001-2009
118
21 (17.8%)
97 (82.2%)
Chromosome 22q11 deletion (pts) 36 (30.5%)
Median age at 1st treatment:
at unifocalization:
11.4 mos
(0.3 - 172)
15 mos
(1 - 419)
Median weight at 1st treatment:
at unifocalization:
7.7 kg
(2.5 - 56 kg)
8.6 kg
(2.9 - 59 kg)
AP + DIV + Collaterali
Prognosi OPBG
Dr. A.Carotti et all (OPBG)
65. 1 year: 86.4 % (95%CI: 78.4-91.6)
5 and 10 years: 83.9 % (95%CI: 75.3-89.7)
Rehabilitation Unifocalization Total
Study Parameter (n=32) (n=86) (n=118)
Single-stage complete repair 56% 67% 64%
Ultimate complete repair 75% 82% 80%
Alive at 4 years’ follow-up 87% 81% 84%
AP + DIV
Prognosi OPBG
Dr. A.Carotti et all (OPBG)
66. Honjo 2009 20 18 0.63 15 0.53 2.5
AP + DIV
Prognosi OPBG
Carotti et all, J Thorac Cardiovasc Surg. 2010 Nov;140(5):1092-103.
67. F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Complesse
Cuori Univentricolari
(Funzionalmente)
6/81 (ogni 10000 nati vivi)
(8%) tutte CC
68. 1. Neonatale
2. Glenn dopo qualche mese
3. Fontan dopo 2-3 anni
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Univentricolare
Trattamento
Palliazione secondo Fontan
3 Stadi
69. Modulazione flusso Polmonare
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Univentricolare
Trattamento
Se Troppo Bendaggio AP
Se Poco Shunt vs Stent duttale
1 Stadio
Cirolazione Sistemica normale
(Non ostacolo al flusso sistemico)
Stent Dotto
Shunt
Bendaggio
70. Atresia Aortica (HLHS – HLHS like)
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Univentricolare
Trattamento
1. Fornire flusso sistemico
2. Modulare flusso Polmonare
1 Stadio
I stadio Norwood vs I stadio Ibrido
73. Norwood II stadio (Glenn)
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Univentricolare
Trattamento
Ibrido 2° Stadio (Glenn)
(Comprehensive)
74. Norwood III stadio (Fontan)
F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Univentricolare
Trattamento
75. F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore funzionalmente
Univentricolare
Prognosi
Cuore sinistro Ipoplasico/like
S. Shone
Canale con Dominanza dx
o Canale AV parziale
o S. Asplenica
o CC Congenita con atresia aortica
Unico ventricolo di morfologia dx
4/6 Univentricolari (ogni 10000 nati vivi)
(2/3 Cuori Univentricolari)
76. F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore funzionalmente
Univentricolare
Prognosi
Cuore destro Ipoplasico
Atresia della Tricuspide
Atresia della Polmonare setto intatto
Doppia entrata ventricolare
Unico ventricolo di morfologia sn
2/6 Univentricolari (ogni 10000 nati vivi)
(1/3 Cuori Univentricolari)
78. F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Funzionalmente Univentricolare
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346
79. F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore Funzionalmente Univentricolare
Prognosi
Gunnar Erikssen et al. Circulation. 2015;131:337-346
80. F.Up delle Cardiopatie Congenite - G.Rinelli -
Cuore sn Ipoplasico vs Cuore dx Ipoplasico
Prognosi
Yves d’Udekem et al. JACC Vol. 59, No. 13, 2012
Melbourne 1990-2008
Palliazione Univentricolare
(499 pts)
81. F.Up delle Cardiopatie Congenite - G.Rinelli -
Ibrido vs Norwood I
Prognosi
Photiadis J et all Thorac Cardiovasc Surg. 2012 Apr;60(3):181-8
82. F.Up delle Cardiopatie Congenite - G.Rinelli -
Ibrido vs Norwood I
Prognosi
Baba K et all Circulation 2012 Sep 11;126(11 Suppl 1):S123-31
Toronto 2004-2010
Palliazione Univentricolare
(110 pts)
75 pts arrivati a Stage II
83. F.Up delle Cardiopatie Congenite - G.Rinelli -
Ibrido vs Norwood I
Prognosi
Baba K et all Circulation 2012 Sep 11;126(11 Suppl 1):S123-31
Toronto 2004-2010
Palliazione Univentricolare
(110 pts)
75 pts arrivati a Stage II
84. F.Up delle Cardiopatie Congenite - G.Rinelli -
Ibrido vs Norwood I
Prognosi
Alexander A Brescia et all J Thorac Cardiovasc Surg. 2014 Jun;147(6):1777-82
2007-2014
40 pts
St. Louis USA
85. F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza dopo la Fontan OPBG
Prognosi
Salvatore Giannico et all JACC Vol 47,n 10,2006
Sopravvivenza
(193 pts)
85% a 15 anni
77% NYHA I
19% NYHA II
3% NYHA III
86. F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza dopo la Fontan OPBG
Prognosi
Salvatore Giannico et all JACC Vol 47,n 10,2006
221 pazienti post
Fontan con condotto
Sopravvivenza
85% a 15 anni
77% NYHA I
19% NYHA II
3% NYHA III
87. F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza dopo la Fontan OPBG
Prognosi
Salvatore Giannico et all JACC Vol 47,n 10,2006
88. F.Up delle Cardiopatie Congenite - G.Rinelli -
Sopravvivenza dopo la Fontan OPBG
Prognosi
Salvatore Giannico et all JACC Vol 47,n 10,2006
89. Conclusioni Come vanno
F.Up delle Cardiopatie Congenite - G.Rinelli -
Prognosi
Cardiopatie Semplici
Benissimo !!!
Correzioni Biventricolari di cardiopatie complesse
Molto Bene!!!
Palliazioni Monoventricolari
Discreto… in miglioramento
c'è da lavorare ancora parecchio !!
(8%) di tutte le CC
Grazie
