Surgery for congenital heart malformations began in Qatar nearly twenty years ago on the 11^th of April, 1983.¹ Initially, this was carried out within the program for acquired heart disease and, for a brief subsequent interval, by visiting specialists from abroad. Since October 2000, there has been the continuous presence of a full-time team dedicated to the care of patients with congenitally malformed hearts. Herein, we review the surgical activity of the past two years and compare it with that of the first ten years, as reported to the GCC Cardiovascular Conference by Dr. Amer Chaikhouni in January 2002.¹

Fig. 1. Age at the time of operation for 296 patients operated between 1983 and 1993, and 150 between 2002 and 2002 at Hamad Hospital.

   Between 1 October 2000 and 30 September 2002, one hundred and fifty patients underwent operation at ages of less than one day to just under 17 years (Figure 1). This represents an increase from about 30 patients per year to 75 cases annually, with a shift of the male: female ratio from 54:46 to 61:39 (Figure 2), in closer alignment with observed gender ratios for congenital heart malformations.2 Fifty-eight (39%) were non-pump procedures, while 92 operations (61%) were done with cardiopulmonary bypass. During this time,

Correspondence: Dr. Roxane McKay, Hamad Hospital,
P.O. Box 3050, Doha, Qatar. Telephone: (974) 439-2584.
Fax: (974) 439-2324. e-mail: rmck07@yahoo.com.

Fig. 2. Congenital cardiothoracic surgery at Hamad Hospital from 1983 to 1993, and from 2000 to 2002. Abbreviation: N = number of procedures.

adult congenital heart patients were managed by the general cardiothoracic surgical team and accordingly are not included in these figures, while a small number of neonates and infants underwent pulmonary procedures on the pediatric cardiac service. Although the proportion of patients older than one year of age remained constant at 50% there was, however, a considerable decrease in the average age at operation from 14.0 to 2.7 years, with more procedures done in the neonatal period.

   Prior to 2000, most neonates and those patients who required complex procedures were sent abroad for cardiac surgery. With more of these cases being done in Doha, Qatari nationals increased to the largest group of patients (41%), compared with 22% between 1983 and 1993 (Figure 3). Nine patients (6%) had genetic syndromes, six received a homograft prosthesis, nine were reoperations, and sixteen (10.6%) had intraoperative transesophageal echocardiography. This latter group ranged in weight from 5.3 Kg to 60 Kg at the time of surgery.

   The most commonly performed procedures during the first ten years were closure of a persistently patent arterial duct (25%), closure of an atrial septal defect (23%), systemic-pulmonary shunt operations (17%), pulmonary artery banding (7%), and complete correction of Fallot’s tetralogy (7%) (Figure 4). By 2000, most atrial septal defects

Fig. 3. Nationality of pediatric cardiothoracic surgical patients.

and patent arterial ducts were being closed with devices in the cardiac catheterization laboratory,3 such that these conditions made up only 3% and 10%, respectively, of the more recent case load. The number of shunt procedures (9%) and pulmonary artery bandings (3%) also decreased as a reflection of the trend towards primary definitive repair in preference to preliminary palliation, while closure of isolated or multiple ventricular septal defects (10%) and repair of coarctation of the aorta (9%) were done more frequently.

Fig. 4. Most commonly performed operations, shown as a percentage of the total procedures for two surgical eras at Hamad Hospital. The "shunt" category included various types of systemic-pulmonary anastomosis, and “tetralogy” referred to both primary and secondary complete correction. Nearly all patients in the "other" group were complex procedures. Abbreviations: PDA = persistently patent arterial duct, ASD = atrial septal defect, PA Band = pulmonary artery banding, VSD = closure of ventricular septal defect.

   The greatest increase, however, was among complex cardiac malformations, which accounted for nearly all of the procedures listed in the "other" category. There is not yet complete consensus regarding a system of risk stratification in congenital heart surgery, but it is nonetheless recognized generally that some procedures - especially complex lesions - and some patients make greater demands than others upon institutional resources and the skills of the surgical team. Using the complexity scores which have been provisionally suggested by the European Congenital Heart Surgeons Foundation,4 and combining the “difficult” and “complex” categories into one group, 47% of all procedures undertaken between 2000 and 2002 were complex (Figure 5).

Fig. 5. Distribution of patients according to complexity of procedure for operations done between 2000 and 2002, using complexity scores of the European Congenital Heart Surgeons Foundation4 for risk stratification.

   These included the arterial switch operation for simple and complex transposition of the great arteries, repair of totally anomalous pulmonary venous drainage, Fontan operations, repair of complete atrioventricular septal defect, and correction of complex pulmonary atresia.

   Overall, there was no statistically significant difference in survival following either open or closed heart operations during the 1983-1993 era and the more recent 2000-2002 experience (Table 1). However, for lesions, which correspond approximately to the case-mix of the first ten years, mortality among open-heart operations decreased notably from 13% to 0%. The results for most specific procedures also compare favorably with

Table 1. Types of procedures and results of surgery

Table 2. Comparison of surgical results at Hamad Medical Corporation, 2000-2002 (HMC) with those reported from the European Cardio-Thoracic Surgical Registry (EUR).5 Abbreviations: ASD = atrial septal defect, VSD = ventricular septal defect, TOF = complete repair of tetralogy of Fallot, CAVSD = repair of complete atrioventricular septal defect, N = number of cases, M = mortality.

Fig. 6. Surgical activity and mortality from October 2000 to September 2002. Numbers on the vertical axis refer to the number of cases operated each month, and letters on the horizontal axis to the month of the year.

those reported from the Pediatric European Cardiothoracic Surgical Registry for 20015 (Table 2). Viewed over time (Figure 6), there has possibly been some improvement in overall patient survival during recent months.

Academic activity is recognized as an important component of the surgical program, both for the advancement of clinical work and for continuing professional development. Surgical members of the team made a number of presentations and

Table 3. Publications and presentation by the surgical team, 2000 – 2002.

publications during the past two years, and the surgical program fostered research and teaching in related disciplines (Table 3).

   COMMENT

   Clinical services tend to reflect the circumstances in which they are provided as well as the patents whom they serve and the individuals who participate in their delivery. As a comparatively isolated, low volume institution without formal collaborative relationships with quaternary centers,6 the challenges faced by the congenital heart surgical program have been considerable. While some Qatari nationals were able to access centers abroad for treatment, this option was not available to most expatriate patients who, in addition, often had limited or no possibility of reoperation or extensive follow-up after returning to their own countries. The general philosophy adopted by the surgical team was to offer operation to all patients who were referred for treatment, recognizing the increased risk of some procedures, and to try to employ techniques which would not commit the patient to anticoagulation or reoperation where this was not available. Thus, for example, Fontan operations were done with direct, extracardiac cavopulmonary connections7 whenever possible, pedicled pericardial valved conduits8 rather than homograft valves have been used for some right ventricle-to-pulmonary artery connections, and classical Blalock-Taussig shunts have been substituted for prosthetic grafts in patients thought to be at increased risk of shunt occlusion.

   Recent reports from both North America9 and the United Kingdom10, 11 have addressed various aspects congenital cardiac services and in particular the relationship between the volume of surgical procedures and outcomes. Elegant statistical analysis suggests that mortality in open heart surgery for patients less than one year of age is inversely related to the number of operations done in a given unit, 11 but, at the same time, recommendations for overall minimum volumes vary from 50 to 300 cases/year. While the results of this program are broadly in keeping with those achieved in busier units, there can be little doubt that a larger caseload would expedite the institutional learning curve, use resources more efficiently, and reduce surgical risk. Theoretically, each additional open-heart operation could decrease the odds of surgical death between 0.20% and 0.58%.11 An increase from approximately 50 open-heart operations to 75 per year would potentially reduce surgical mortality by 10% to 15%, depending upon the specific types of procedures. A high priority is thus to achieve an annual caseload of about 100 congenital procedures per year.

   The overall aspiration of the surgical program remains to provide a safe, sustainable and comprehensive service to all patients with congenital heart disease, through the acquisition and development of the relevant infrastructure as well as accumulation of expertise and experience by all members of the team. Continued turnover of personnel in a migratory work force from widely divergent backgrounds has made this a complicated mission, and it is likely that optimal stability of the program will be reached only as more permanent residents assume positions of responsibility and leadership. However, considerable progress has been made towards this goal, and this reflects enormous efforts on the part of many individuals and departments throughout the Hamad Medical Corporation. These are gratefully acknowledged.

References

1. Chaikhouni A. Cardiac operations for congenital heart disease in Qatar: the first ten years. Heart Views 2002;2:168-169.

2. Rosenthal G, Prevalence of congenital heart disease. In: Garston A Jr, Bricker JT, Fisher DJ, Neish SR (eds). The Science and Practice of Pediatric Cardiology, Second Edition. Williams & Wilkins, Baltimore, 1998, pp 1083-1105.

3. Robida A, Bricelj B, Kohli V. Closure of the arterial duct in the catherization laboratory. Heart Views 2002;2:167.

4. Lacour-Gayet F. Risk stratification theme for congenital heart surgery. Semin Thorac Cardiovasc Surg 2002;5:148-152.

5. Maruszewski B, Tobota Z. The European congenital heart defects surgery database experience: Pediatric European Cardiothoracic Surgical Registry of the European Association for Cardio-Thoracic Surgery. Semin in Thorac Cardiovasc Surg 2002;5:143-147.

6. Neirotti R, Malclom D, Sanfilippo D, Jones D, Fosse G, Steffens T. Pediatric cardiac surgery in a low volume institution: Quality assessment (Abstract). American Academy of Pediatrics, Washington, DC, October 1999.

7. McKay R, Kakadekar AP, Tyrrell MJ. Extracardiac Fontan operation: Direct cavopulmonary connections. Cardiology in the Young 1998;8:274.

8. Iemura J, Oku H, Otaki M, Kitayama H, Matsumoto T. Reconstruction of right ventricular outflow tract by pedicled pericardial valved conduit. Ann Thorac Surg 1997;64:1849-1851.

9. Sinclair M. The report of the Manitoba Pediatric Cardiac Surgery Inquest: An inquiry into twelve deaths at the Winnipeg Health Sciences Center in 1994. http.//www.pediatriccardiacinquest.mb.ca

10. Report of the pediatric and congenital cardiac services review group. http.//www.doh.gov.uk/childcardiac/ reviewnov02.pdf

11. Spiegelhalter DJ. Mortality and volume of cases in paediatric cardiac surgery: retrospective study based on routinely collected data. BMJ 2001;323:1-5.