All posts in neonatal cardiology

June 2013 Parma Echo Meeting From Fetus to Joung Adult

Dear colleagues and friends,

here is the Program of our June 2013 Parma Echo Meeting.

We invite you to partecipate to “…a very special and unique Meeting ! …“

“ .. Avec un programme scientifique de qualité et une hospitalité unique,

prenant soin de toute chose, de tous et de chacun!… “

“ L’atmosfera come sempre e’ affettuosa e charming …”

“ The Parma Meeting continues to have a unique place in our lives !”

Vi aspettiamo ! per un’altra “ wonderful experience ! “

M Ludovica Maramotti Prezioso Umberto Squarcia

Max Mara Universita’ di Parma


Centro S.Elisabetta Campus Universitario


Structural Heart Diseases in Young Adult

9:00 – 9:30 Advanced 3D Imaging of Right Ventricle. Morphology and Function L Badano
( Padova , Italy )

9:30 – 10:00 Case Examination

10:00 – 10:30 Echocardiografic Assessment of Coronary Reserve F Rigo (Venice, Italy )

10:30 – 11:00 Case Examination

11:00 – 11:30 Coffee Break

11:30- 12:00 Rest Speckle Tracking in Coronary Arteries Diseases N. Gaibazzi

( Parma ,Italy )

12:30 Lunch


14:00 – 14:15 Echocardiographic Assessment of RV Function M Al-Obeyde ( Mosul, Iraq )

14:15- 14:45 New Echocardiographic Methods to evaluate the RV Function M Vogel
( London, UK )

14:45 – 15:00 Pulmonary Hypertension in CHD. An Overview. A Agnetti ( Parma, Italy )

15:00 – 15:30 Current Treatment of PH Associated With CHD N Galiè ( Bologna, Italy )

15:30 – 16:00 Echocardiography in the Management of Pediatric Pulmonary Hpertension
J Johnson ( Rochester , Minn. USA )

16:00 – 16:30 Panel and General Discussion

Aula Magna, Università degli Studi di Parma

18:30 – 19:30 Opening Ceremony

Rastelli Lecture by Norman Silverman ( S.Francisco, USA )
The History of Pediatric Echocardiography Where We Have Come and Where We Are Going!

20:00 – 22:00 Reception and Dinner at St. John Evangelist Church’s Refectory



9:00 – 9:30 Embryological development of the heart –S Sanders ( Boston, Mass. USA )

9:30 – 10:00 Fetal detection of Congenital Heart Diseases L Fermont ( Paris, France )

10:00 -10:30 The future of 3D Fetal Echocardiography N Silverman ( S.Francisco , Cal. USA )

11:00 – 11:30 Coffee Break

11:30 – 12:00 “ Heaven can wait ! “ A Modern Vision of Fetal and Perinatal Cardiology

( Project born inside the Parma’s Gang ) M Guirgis ( Paris, France )

12:00 – 12:30 Isabelle Jue Lecture by Paul Julsrud ( Rochester, Minn. USA )

Anomalous Coronary Artery Anatomy –Revisited

12:30 Lunch

14:00 – 14:30 Post Mortem Imaging S Sanders ( Boston, Mass. USA )

14:30 – 15:00 Fibrosis, Cirrhosis or Worse: What Happens to the Liver in the Fontan
Circulation J Johnson ( Rochester, Minn. USA )

15:00 – 17:00 Case Examination

19:00 – 23:00 Max Mara Gala Dinner in Reggio Emilia


MRI/CT/3D/Pathologic Correlation

9:00 – 9:20 MRI / CT / Pathology Correlation in Intracardiac Anatomy S Sanders
( Boston, Mass. USA )

9:20 – 9:30 3D Reconstruction of heart specimen S Sanders ( Boston, Mass. USA )

9:30 – 10:00 3D for RV Function G Shirali ( Kansas City , Miss. USA )

10:00 – 10:30 MRI / CT / Pathology Correlation in Vascular Rings S Sanders
( Boston, Mass. USA )

10:30 – 11:00 Coffee Break

11:00 – 11:30 MRI late FU in PO Fallot patients P Festa ( Pisa, Italy )

11:30 – 12:00 How do Echocardiographic Measures of Ventricular Function match up with

Catheter Measures in CHD. G.Shirali ( Kansas City , Miss. USA )

12:00 – 12.30 Panel Discussion ( Hagler, Sanders, Silverman, Shirali, Julsrud, Festa )

12:30 Lunch

14:00 – 14:30 Conventional Measures of LV Function : Implications of the Pediatric Heart

Network Studies G Shirali ( Kansas City , Miss. USA )

14:30 – 15:00 Catheter Interventional Procedures in ACHD D Hagler
( Rochester, Minn. USA )

!5:00 – 17:00 Case Examination

Aula Magna, Universita’ degli Studi di Parma

18:00 – 19:00 Premio Vincenzo Ferrante for young scientists
Chairperson R. Williams ( Los Angeles Cal. USA )

Macartney Lecture by Fernando and Madalena Maymone Martins
( Lisboa, Portugal )

“Helping people in difficulty – A view within and
beyond Pediatric Cardiology

20:00 – 23:00 Dinner and Music in the Countryside

“ Under the Stars of Mamiano “

The Accompanying Persons will be guided to visit the sites of Parma and Province

by Umberto Squarcia Jr., Architect in New York, Cortina and Parma


Post Congress Tour

Umberto Squarcia MD FACC

Full Professor of Pediatrics and

Pediatric Cardiology

University of Parma Italy

Past Member of Board of Directors

Mayo Alumni Association

Rochester,Minn. USA

Cell. +39 338 6035868


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Bicuspid Aortic Valve Disease

Bicuspid aortic valve disease (BAV) is the most common congenital heart abnormality, affecting 0.5–2% of the total population with a male predominance [1]. Although the majority of cases are sporadic, BAV can be familial and is commonly associated with other congenital heart abnormalities and genetic syndromes [1,2]. In addition to the obvious valvular sequelae to which BAV patients are predisposed, BAV disease is associated with an aortopathy and an associated predisposition to aneurysm formation and dissection of the thoracic aorta [1–3]. Patients with BAV therefore require lifelong evaluation and surveillance at various intervals, depending upon their specific condition. Advances in imaging and in molecular characterization of the underlying aortopathy hold promise to improve both evaluation and treatment of patients with BAV.
The defining feature of BAV is abnormal aortic valvular morphology, with the valve having two leaflets and two commissures instead of three. Most commonly, the two leaflets are asymmetric, and the larger has a central raphe or fibrous ridge in the area where a commissure that would otherwise be present has fused [1]. The leaflets may be of roughly equivalent size, and the raphe may be absent in certain cases [1]. Fusion may occur between any of the three valve leaflets, most commonly between the right- and left-coronary leaflets (70–86%), although also between the right- and non-coronary leaflets (12%) or between the left- and non-coronary leaflets (3%) [1]. Leaflet orientation may play a role in BAV disease progression and aortic dilatation [2,4]. Right- coronary and non-coronary leaflet fusion has been associated with more rapid progression of valvular pathology including stenosis and re- gurgitation [4]. The abnormal hemodynamic stress imposed by the atyp- ical valve anatomy on both the valve tissue and the aorta by BAV may contribute significantly to the ultimate development of valvular dysfunction and to aortopathy [2,5,6].

BAV patients may have many fates. Some patients with BAV are highly symptomatic in childhood with congenital AS or severe AR or have associated lesions such as coarctation of the aorta, which bring them to medical attention. However, many young people with BAV are completely asymptomatic and have “normally” functioning BAVs. It is nevertheless estimated that at least one third and perhaps a majority of patients with BAV will develop a complication during their lifetime. This implies that the vast majority of BAV patients will remain asymptomatic until adulthood, at which time they begin to experience progressive degeneration of valvular function and sequelae of aortopathy including aortic dilatation, aneurysm formation and risk of dissection. Early surgical and autopsy data indicate that progression of valvular dysfunction, particularly aortic stenosis, occurs at a significantly earlier age in BAV patients than in those with a trileaflet aortic valve.

The abnormal hemodynamic stress imposed by the atypical valve anatomy on both the valve tissue and the aorta by BAV may contribute significantly to the ultimate development of valvular dys- function and to aortopathy


[1] BravermanAC,GuvenH,BeardsleeMA,MakanM,KatesAM,MoonMR. The bicus-pid aortic valve. Curr Probl Cardiol 2005;30(9):470-522.

[2] Braverman AC. Beardslee. The bicuspid aortic valve. In: Otto C, Bonow R, editors. Valvular heart disease: a companion to Braunwald’s Heart Disease. Philadelphia: Saunders/Elsevier; 2009. p. 169-86.

[3] Braverman AC. Aortic involvement in patients with a bicuspid aortic valve. Heart 2011;97(6):506-13.

[4] Fernandes SM, Khairy P, Sanders SP, Colan SD. Bicuspid aortic valve morphology and interventions in the young. J Am Coll Cardiol 2007;49(22):2211-4.

[5] Wallby L, Janerot-Sjoberg B, Steffensen T, Broqvist M. T lymphocyte infiltration in non-rheumatic aortic stenosis: a comparative descriptive study between tricuspid and bicuspid aortic valves. Heart 2002;88(4):348-51.

[6] Robicsek F, Thubrikar MJ, Cook JW, Fowler B. The congenitally bicuspid aortic valve: how does it function? Why does it fail? Ann Thorac Surg 2004;77(1):177-85.

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7th European Echocardiography Course in Congenital Heart Disease

The 7th European Echocardiography Course in Congenital Heart Disease will be organised in

Barcelona, from Wednesday 3th to Saturday 6th October 2012.
This course provides a thorough overview of Echocardiography in Congenital Heart Disease and is aimed at Pediatric Cardiologists, Grown-up Congenital Heart Disease Specialists and Echocardiography Technicians working with Congenital Heart Disease patients.

The programme strongly builds on the side-by-side comparison of morphology and imaging and discusses the major abnormalities found in clinical practice. The course prepares for the accreditation exam for ‘Congenital Heart Disease Echocardiography’ organised by the EAE/AEPC.

Additionally, we will organise the 1st Symposium on Fetal Cardiac Function in conjunction with the EEC in Barcelona, on Tuesday 2nd of October 2012.

In this symposium, the basic principles of cardiac function and deformation will be explained and the techniques for measuring this in the fetus will be presented and discussed by experts in the field. Additionally, the changes in pathological situation will be highlighted.

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6th World Congress of Paediatric Cardiology and Cardiac Surgery 2013

The 6th World Congress of Paediatric Cardiology and Cardiac Surgery 2013 takes place from the 17th to the 22nd February 2013.
The World Congress is run for and on behalf of the International Steering Committee for the World Congress and builds on the solid scientific foundations established at the previous World Congresses.

We are putting together a first class 5 day program for everyone involved in the care of children with all forms of heart disease.

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Chiari Network as a Cause of Fetal and Neonatal Pathology

Chiari’s Network as a Cause of Fetal and Neonatal Pathology
Fatiha Bendadi • David A. van Tijn • Lou Pistorius • Matthias W. Freund

Keywords Additional heart sound  Chiari’s network 
Congenital heart disease  Fetal hydrops

Received: 17 June 2011 / Accepted: 26 August 2011

Open Access This article is distributed under the terms of the
Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any
medium, provided the original author(s) and source are credited.

The Author(s) 2011. This article is published with open access at

Chiari’s Network as a Cause of Fetal and Neonatal PathologyAbstract Chiari’s network is a remnant of the eustachian valve located in the right atrium.

Chiari’s Network as a Cause of Fetal and Neonatal PathologyAbstract Chiari’s network is a remnant of the eustachian valve located in the right atrium. Incomplete involution of the fetal sinus venosus valves results in ‘‘redundant’’ Chiari’s network, which may compromise cardiovascular function. This report describes a case with the novel finding of prenatal compromise due to redundant Chiari’s network and an uncommon case with significant postnatal symptoms. In both cases, the symptoms (fetal hydrops and postnatal cyanosis) resolved spontaneously. The variety of cardiovascular pathologies described in the literature is believed to be associated with persistence of a Chiari network. Knowledge about this not always harmless structure is important for perinatologists, pediatricians, and pediatric cardiologists alike. The clinical importance of this rare
pathology is that prenatal counseling may anticipate a generally positive outcome and that surgical intervention generally should be avoided.

Received: 17 June 2011 / Accepted: 26 August 2011
The Author(s) 2011. This article is published with open access at

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46th Annual Meeting of the Association for European Paediatric and Congenital Cardiology

Dear Friends and Colleagues,

Dear Friends and Colleagues, It is our pleasure to invite you to the 46th Annual Meeting of the Association for European Paediatric and Congenital Cardiology which will be held in Istanbul. We are pleased to organise this wonderful event for the second time in Istanbul after the 1976 meeting of AEPC. We believe this meeting will give you the opportunity to increase the knowledge exchange in paediatric and congenital cardiology. The meeting will cover all the aspects of this unique medical science.

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Fetal and Pediatric Cardiology Seminar in Paris

Fetal and Pediatric Cardiology Seminar in Paris
December 9th and 10th of 2011 – Paris – FRANCE

Dear Friends,

We propose this Seminar on fetal and pediatric cardiology. We meant it to be different. Indeed, the presentations will be delivered by Junior / Senior pairs. We hope you will find appealing and exciting this array of subjects ranging from genetics, embryology, first quarter imaging, 3D and 4D imaging and magnetocardiography to pathophysiology of the coronary flow and neurodevelopment, pathologies of the left side of the heart and surgical procedures.
We will usher you into a recent though highly innovative disciplines, with both the experience of the greatest international experts and the enthusiasm of junior practitioners, tomorrow’s seniors, to escort us. We will be speaking English: it is the language of scientific discourse, used in both debate and publications,but with available translation into French.



you can also copy it on google and save it in your favourites

hope to see you in Paris

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Kabuki Syndrome

Kabuki Syndrome
a post_it by s.gerboni

Kabuki syndrome, also known as Kabuki makeup syndrome and Niikawa-Kuroki syndrome, was initially described in 1988 by Niikawa et al. Congenital heart disease is commonly associated with Kabuki syndrome, with a reported incidence of 31–58% in large series

Most patients with Kabuki syndrome have five cardinal features:

  1. distinct facial features,
  2. postnatal growth retardation,
  3. developmental delay or mental retardation,
  4. skeletal abnormalities,
  5. and dermatoglyphic abnormalities.

The diagnosis is determined clinically because the chromosomes are normal
and no clinical test exists to confirm the clinical diagnosis of Kabuki syndrome.
The association of cardiac defects with Kabuki syndrome has been well described. The majority of these
defects are isolated shunt lesions, conotruncal abnormalities, or various forms of arch obstruction.
The spectrum of associated cardiac defects is varied.
The finding of left-sided obstructive lesions, specifically coarctation of the aorta, is reported in up to 29% of cases and in my serie three patients have a Shone Sindrome. Only two cases of associated congenital heart disease with single-ventricle physiology have been reported.
Recently were reported a case series of three patients with Kabuki syndrome and single-ventricle physiology, specifically hypoplastic left heart syndrome (HLHS), from a single institution This complete and illustrates the full spectrum of left-sided obstructive lesions and expands the phenotype of cardiac defects associated with Kabuki syndrome.

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