Ra-id Abdulla

Multicenter Experience with Perventricular Device Closure of Muscular Ventricular Septal Defects

Hybrid procedures are becoming increasingly important, especially in the management of congenital heart lesions for which there are no ideal surgical or interventional options. This report describes a multicenter experience with perventricular muscular venticular septal defect (VSD) device closure.Three groups of patients (n = 12) were identified: infants with isolated muscular VSDs (n = 2), neonates with aortic coarctation and muscular VSDs (n = 3) or patients with muscular VSDs and other complex cardiac lesions (n = 2), and patients with muscular VSDs and pulmonary artery bands (n = 5). Via a sternotomy or a subxyphoid approach, the right ventricle (RV) free wall was punctured under transesophageal echocardiography guidance. A guidewire was introduced across the largest defect. A short delivery sheath was positioned in the left ventricle cavity. An Amplatzer muscular VSD occluding device was deployed across the VSD. Cardiopulmonary bypass was needed only for repair of concomitant lesions, such as double-outlet right ventricle, aortic coarctation, or pulmonary artery band removal. No complications were encountered using this technique. Discharge echocardiograms showed either mild or no significant shunting across the ventricular septum. At a median follow-up of 12 months, all patients were asymptomatic and 2 patients had mild residual ventricular level shunts. Perventricular closure of muscular VSDs is safe and effective for a variety of patients with muscular VSDs.

Use of intracardiac echocardiography to guide catheter closure of atrial communications.

Intracardiac echocardiography (ICE) is slowly replacing transesophageal echocardiography as the preferred imaging tool to guide device closure of atrial septal defects and patent foramen ovale. This article is a brief review of the literature related to ICE, the technical aspects ICE imaging, techniques for obtaining the standard views, and the future directions of this methodology. (ECHOCARDIOGRAPHY, Volume 20, November 2003)

Aortic Stenosis: The Spectrum of Practice

There is significant variation in practice patterns in managing congenital aortic valve stenosis. Review of medical literature reveals no significant information regarding the current practice methods in the treatment of a simple lesion such as aortic stenosis (AS). Therefore, this survey-based study was conducted in an attempt to better understand the uniformity or heterogeneity of practice in treating AS. A questionnaire was prepared to evaluate the style of management of AS. This survey was designed to assess the practice of follow-up visitations, type and frequency of investigative studies, pharmacological therapy, and exercise recommendations. Questions about therapeutic intervention included those of timing and type of intervention. Questionnaires were sent to all academic pediatric cardiology programs in the United States (48 program) and selected international programs from Europe, Asia, and Australasia (19 program). The total number of surveys sent out was 67, and the total number of respondents was 25 (37%), 15 (31%) from the United States and 9 (53%) from outside the United States. The definition of moderate AS varied among respondents. The range provided for mild AS was identified as that with a peak-to-peak pressure gradient of < 25–30 mmHg, peak instantaneous Doppler gradient of < 36–50 mmHg, or mean Doppler gradient of < 25–40 mmHg. On the other hand, severe AS was defined as that with a peak-to-peak gradient of > 50–60 mmHg, peak instantaneous Doppler gradient of > 64–80 mmHg, or mean Doppler gradient of > 45–64 mmHg. In assessing follow-up patterns, 84% of respondents recommended seeing patients with mild AS annually, the longest time of follow-up listed in the questionnaire, whereas 20% suggested follow-up every 6 months. There was no consensus among survey centers regarding follow-up of patients with moderate AS. For severe AS, 16% recommend immediate intervention, 16% arrange follow-up every 6 months, and 56 and 28% recommend follow-up in 3 and 1 month(s), respectively. In making the decision to proceed with biventricular versus univentricular repair in patients with AS in the neonatal period, many factors were considered. Ninety-two percent of respondents rely on mitral valve z score, 84% on aortic valve z score, 52% on left ventricle length, 48% on the presence of antegrade ascending aorta flow, and only 32% considered significant endocardial fibroelastosis as a factor. Rhodes score was used by 20% of respondents in decision making regarding the approach to management of this subset of AS. This study shows that there is consensus in the management of mild and severe forms of AS. As expected, disagreement is present in the definition, evaluation, and therapy of moderate aortic valve stenosis. There is a tendency for catheter intervention except in the presence of dysplastic aortic valve or moderate to severe aortic regurgitation. There is also disagreement regarding methods used to determine biventricular versus univentricular repair of a borderline hypoplastic left heart.

Atrial Septal Defect: Spectrum of Care

Atrial septal defect (ASD) is a common congenital heart defect. Variability in management of this lesion exists among clinicians. A review of the literature reveals that there is lack of standard guidelines for the evaluation and management of patients with different types of ASDs. This survey-based study was conducted to test the uniformity of diagnostic and therapeutic approach to management of children with secundum, sinus venosus, and primum ASDs. Survey questionnaires were prepared to include questions regarding follow-up, diagnosis, and therapeutic intervention of different types and sizes of ASDs. Questions addressed follow-up visitations, type and frequency of investigative studies, pharmacological therapy, and choice of repair method. Surveys were sent out to all pediatric cardiology academic programs in the United States (n=48) and randomly selected international programs from Europe, Asia, and Australia (n=19). A total of 23 programs (34%) responded to the survey (15 from the United States and 8 internationally). A separate questionnaire was prepared for secundum, primum, and sinus venosus ASD. In each questionnaire, lesion types were subdivided into small, moderate, and large defect sizes to address differences of management approaches to each defect type and size. Results indicate that in secundum ASD, most participants use size of the defect and/or evidence of right-sided volume overload as criteria for defining small, moderate, and large defects. Frequency of follow-up does not vary with the type of lesion but is more frequent with larger defects. Most participants see patients with small defects at intervals of 6 months to 1 year and those with large defects at 3- to 6-month intervals. Age of patient and presence of symptoms determined the frequency of follow-up across all defects. Echocardiography was the most frequently used investigative modality in all defect sizes and types during follow-up visits (used by >80% for follow-up), followed by electrocardiography (ECG). There is a striking preference for the use of pharmacological therapy in primum ASD compared with secundum and sinus venosus ASD. The timing of repair was mainly dependent on patient age and symptomatology in different defects, with the presence of associated anomalies contributing to that in primum and sinus venosus ASD. Most participants use percutaneous approach to close secundum ASD (either as a first choice or as one of two choices depending on the presence of certain features). Before repair, participants use MRI or cardiac catheterization to fully evaluate a secundum ASD if it is large. These investigative modalities are not commonly used in primum and sinus venosus ASD. There is agreement on postoperative follow-up in different types of defects, with most participants continuing follow-up indefinitely, especially in larger defects.

The Pediatric Cardiology Pharmacopeia: 2013 Update

The use of medications plays a pivotal role in the management of children with heart diseases. Most children with increased pulmonary blood flow require chronic use of anticongestive heart failure medications until more definitive interventional or surgical procedures are performed. The use of such medications, particularly inotropic agents and diuretics, is even more amplified during the postoperative period. Currently, children are undergoing surgical intervention at an ever younger age with excellent results aided by advanced anesthetic and postoperative care. The most significant of these advanced measures includes invasive and noninvasive monitoring as well as a wide array of pharmacologic agents. This review update provides a medication guide for medical practitioners involved in care of children with heart diseases.

What Is the Prevalence of Congenital Heart Diseases

26. Trowitsch E, Colan SD, Sanders SP (1985) Two-dimensional echocardiographic estimation of right ventricular area change and ejection fraction in infants with systemic right ventricle (transportation of the great arteries of hypoplastic left heart syndrome). Am J Cardia! 55:1153-1157 27. Vandenberg BF, Rath LS, Stuhlmuller P, Melton HE Jr, Skorton DJ (1992) Estimation of left ventricular cavity area with an on-line, 28. Vogel M, White A, Redington AN (1995) In vitro validation of right ventricular volume measurement by three dimensional echocardiography. Br Heart J 74:460-463 29. Wann LS, Stickels KR, Bamrah VS, Gross CM (1984) Digital processing of contrast echocardiograms; a new technique for measuring right ventricular ejection fraction. Am J Cardia! 53:11641168 30. Williams WG, Trusler GA, Kirklin JW, eta!. (1988) Early and late results of a protocol for simple transposition leading to an atrial switch (Mustard) repair. J Thorac Cardiovasc Surg 95:717-726

A statement on ethics from the HEART Group.

Over the past several years, the editors of leading international cardiovascular journals have met to form the HEART group and to discuss areas of growing, common interest. Recently, the HEART group has developed a document that addresses general ethical principles in the conduct of the scientific process with which all of the editors concur. Published essentially simultaneously in all of the participating journals, including this journal, this document presents the ethical tenets accepted by all of the undersigned editors that will (continue to) guide their decisions in the editorial process. These are the general principles on which the HEART Group is based and by which we, as a group, abide; however, please note that individual journal members and their respective societies may have their own rules and regulations that supersede the guidelines of the HEART Group.

Corrected QT Interval: Reference to QT Interval as a Centile of Normal Range

I. Ades LC, Gedeon AK, Wilson MJ, et al (1993) Barth syndrome: clinical features and confirmation of gene localisation to distal Xq 28. Am J Med Genet 45:327-334 2. Barth PG, Scholte HR, Berden JA, et al (1983) An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes. J Neural Sci 62:327-355 3. Baum D, Bernstein D, Starnes VA, et al (1991) Pediatric heart transplantation at Stanford: results of a 15 year experience. Pediatrics 88:203-214 4. Bolhuis PA, Hensels GW, Hulsebos TJM, Baas F, Barth PG (1991) Mapping of a locus for X-linked cardioskeletal myopathy with neu5. Ibe1 H, Endres W, Hadorn HB, et al (1993) Multiple respiratory chain abnormalities associated with hypertrophic cardiomyopathy and 3-methylglutaconic aciduria. Eur J Pediatr I 52:665-670 6. Ino T, Sherwood WG, Cutz E, et al (1988) Dilated cardiomyopathy with neutropenia, short stature, and abnormal carnitine metabolism. J Pediatr ll3:511-514 7. Kelley RI, Cheatham JP, Clark BJ, et al (1991) X-linked dilated cardiomyopathy with neutropenia, growth retardation, and 3-methylglutoconic aciduria. J Pediatr 119:738-747 8. Neustein HB, Lurie PR, Dahms B, Takahashi M (1979) An X-linked recessive cardiomyopathy with abnormal mitochondria. Pediatrics 64:24-29 9. Tranchant C, Mousson B, Mohr M, eta! (1993) Cardiac transplantation in an incomplete Kearns-Sayre syndrome with mitochondrial DNA deletion. Neuromuscul Disord 3:561-566

The Fontan Procedure: Now What?

The decision to commit a patient with single ventricle to the Fontan procedure route is made every day by pediatric cardiologists and cardiovascular surgeons. At this point, there is no other viable alternative to the palliative procedure we currently refer to as Fontan Procedure, first described by Francois Fontan in 1971 [1], yet more than four decades later there is concern regarding the long-term outcome of patients with Fontan circulation [4, 11, 13]. Specialists in our field will agree regarding the wisdom of choosing the Fontan procedure for patients with single ventricle (anatomical or physiological due to inability to partition), the alternative is a short and complication fraught life of a systemic-pulmonary arterial shunt. Cardiac transplantation is recommended by a limited number of specialists in this field and deemed by most a fate worse than that encountered through lifelong Fontan circulation and reserved by most cardiologists for those with failed Fontan circulation. The physiological concept behind the Fontan circulation is simple: allow blood to flow from the systemic veins through the pulmonary circulation and beyond to the systemic (single) ventricle without the aid of a pumping (right) ventricle. The flow of blood from the systemic veins to the systemic ventricle is enabled by elevation of the systemic venous pressure, the single ventricle’s diastolic relaxation, and the hopefully low resistance of the pulmonary circulation in between. This achieves separation of the two circulations, thus eliminating cyanosis. It also underloads the single ventricle which after completion of the Fontan procedure would have to deal with the systemic cardiac output alone, therefore, reducing the chances of pump failure. Many Fontan patients are alive in their 3 decade of life, but their wellbeing is challenged by serious complications, many are life-threatening [8]. Fontan procedures completed over the past 3–4 decades were not performed as currently done. It is safe to assume that these surgeries will not be done in the future as currently performed. Surgical techniques coupled with anesthesia and post-operative care allowing safer transition to the new anatomy and physiology of the various phases of the Fontan circulation are constantly improving, therefore, comparing results from yesteryears to current ones is rife with inaccuracies. In addition, interventional cardiac procedures in the cardiac catheterization laboratory currently allow us to improve upon what surgery offers and will certainly add to the better outcome for patients with single ventricle. The fruits of this advancement are noted in many studies of this population of patients assessing ventricular function and cardiac arrhythmias [5, 9], however, the outcome of patients with the Fontan circulation continues to be concerning as unique complications plague these patients, such as heart failure with preserved ventricular contractility, thromboembolism, protein-losing enteropathy, and liver failure. [9, 11, 13] The shortcomings of a Fontan circulation includes: (a) reliance on a single ventricle and (b) alteration of central venous and pulmonary arterial pressures and flow patterns. The single ventricle has to endure the added burden of increase pulmonary blood flow for months as well as the insult from repeated surgical procedures but thereafter assumes a normal volume burden; this is usually well tolerated if it is architecturally a left ventricle. A morphological right ventricle on the other hand may suffer from inefficient muscular design and an incompetent tricuspid valve. The alteration of the central venous and pulmonary arterial circulations is most probably more R. Abdulla (&) Section of Pediatric Cardiology, Rush University, Chicago, USA e-mail: Ra-id_Abdulla@rush.edu

A statement on ethics from the HEART Group

Over the past several years, the editors of leading international cardiovascular journals have met to form the HEART Group and to discuss areas of growing, common interest. Recently, the HEART Group has developed a document that addresses general ethical principles in the conduct of the scientific process with which all of the editors concur. Published essentially simultaneously in all of the participating journals, including this journal, this document presents the ethical tenets accepted by all of the undersigned editors that will (continue to) guide their decisions in the editorial process.