2025, Number 4
Ten-year experience in cardiac surgery for congenital heart disease at a high specialty hospital
Full text
10.35366/121356
Language: English
References: 14
Page: 110-115
PDF size: 733.53 Kb.
ABSTRACT
Objective: to describe the initial historical experience of a third-level hospital in surgery for congenital cardiovascular malformations performed by a single surgeon, to present the postoperative results based on their morbidity and mortality, and to compare the preoperative surgical risk with internationally accepted quality standards. Material: a retrospective, analytical, cross-sectional clinical study was carried out, which included pediatric patients diagnosed with congenital heart disease who underwent surgery over a 10-year period. Sociodemographic, diagnostic, therapeutic, morbidity and mortality variables were considered. Results: a total of 112 patients with congenital heart disease were studied. A predominance of females and schoolchildren was observed in this series. The main malformations operated on were patent ductus arteriosus and atrial septal defects. A morbidity of 22% and an overall mortality of 8% were observed. Conclusions: the creation of regional third-level hospitals has promoted the development of pioneering programs in cardiovascular surgery such as the one described in this article, obtaining favorable results comparable with international mortality standards. However, the care of increasingly complex heart diseases remains a challenge to face in the future.ABBREVIATIONS:
- ASD = atrial septal defect
- CPB = cardiopulmonary bypass
- PDA = patent ductus arteriosus
- RACHS-1 = Risk Adjustment in Congenital Heart Surgery -1 Scale
- VSD = ventricular septal defect
Heart defects are the most frequent congenital malformations1 and are the result of an alteration in the heart morphology during intrauterine development2 that lead to a functional impact at birth. The worldwide incidence is 8 to 12 per 1,000 newborns, and they are associated with an increased perinatal and long-term morbidity and mortality.3 Clinical presentation has a wide spectrum that ranges from the absence of symptoms to cyanosis, shock and heart failure. During the first month of life, at least one third of congenital heart defects will require some type of intervention with a high risk of death. They can also be detected in the pediatric stage due to a delay in timely diagnosis.4
Surgery is one of the therapeutic alternatives for congenital heart disease. Although there are several perioperative risk scales, the most widely used due to its practicality is the Risk Adjustment in Congenital Heart Surgery -1 Scale (RACHS-1) developed by Boston Children's Hospital, which classifies 207 surgical procedures into six groups with increasing mortality risk. The procedures with the lowest surgical risk are placed in the first group, while the sixth group contains those with the greatest surgical complexity and highest risk of perioperative mortality.5
Regionalization aims to rationalize medical care based on the population and existing resources, in order to increase the number of treated cases, improve the quality of care and reduce mortality. Based on international recommendations, Mexico requires 21 to 25 specialized medical-surgical centers, of which there are 10, eight of them located in Mexico City, one in Monterrey and one in Guadalajara. The high frequency of congenital heart disease implies the need for more prepared cardiovascular surgical centers.1 Our Institution (Ixtapaluca Regional High Specialty Hospital) is one of the hospitals that was created in the country to meet these decentralization needs, formally beginning its healthcare activities in 2012.6 The objective of this article is to describe the initial historical experience at our Institution in the surgical therapeutic approach to cardiac malformations in pediatric patients, to present the postoperative results based on their morbidity and mortality, and to compare the preoperative surgical risk with internationally accepted quality standards.
MATERIAL
A retrospective, analytical, cross-sectional clinical study was designed including all pediatric patients operated on for congenital heart disease at our Institution from the beginning of its cardiac surgical care activities (2013) until completing the first 10 years of institutional experience by a single surgeon. All pediatric patients with congenital heart disease were included, without distinction of gender or surgical opportunity (elective or emergency surgery). Those patients that were not operated on at our institution in the indicated period were excluded.
Sociodemographic variables, weight, height, diagnosis, need for cardiopulmonary bypass (CPB), postoperative morbidity and mortality, and the preoperative RACHS-1 scale were taken into account. Data were obtained from the institutional electronic medical record and compiled in an Excel spreadsheet in order to subsequently be analyzed with SPSS v.21 statistical software. Numerical variables are presented as mean ± standard deviation, with minimum and maximum variability ranges. Categorical variables are presented as frequency (n) and percentage in relation to the population at risk. Institutional operative mortality was compared with that predicted by the RACHS-1 risk scale using the χ2 test, calculating the Odds Ratio with a 95% confidence interval. A p < 0.05 was considered statistically significant. The study was registered and approved by the institutional research and research ethics committees. Confidential handling measures for the data obtained were followed.
RESULTS
This study presents 112 pediatric patients diagnosed with congenital heart disease operated on at a third-level hospital as an initial experience, exposing the results in the three stages of the surgical procedure: preoperative, intraoperative and postoperative.
PREOPERATIVE RESULTS
Table 1 shows the sociodemographic characteristics, diagnosis according to the type of malformation, preoperative history, and immediate postoperative results. Regarding gender, it can be observed that more than half of the patients were women (female:male ratio of 1.6:1). Almost a third of the patients were schoolchildren, followed by preschoolers and older infants. According to the sequential classification of congenital heart defects, more than half of the pediatric patients were operated on for level 6 malformations (of the large vessels), followed by atrial (level 2) and ventricular (level 4) malformations. All patients were given the RACHS-1 surgical risk scale, classifying them into 6 main risk categories and a complementary category of non-classifiable ones. It can be observed that more than half of the cases were classified in category 1 (73%), and almost a tenth of them were unclassifiable (9%). All patients underwent primary surgery, except for one case that needed re-operation (1%). According to the clinical condition of the heart disease at the time of hospitalization, most of the surgeries performed were scheduled, with less than a quarter of the surgical procedures being urgent. Almost two thirds of the surgeries were performed without CPB and the rest required CPB. It can also be observed that almost all of the surgeries performed were cardiovascular procedures, with those of a thoracopulmonary nature being practically negligible.
INTRAOPERATIVE RESULTS
Table 2 shows the cardiovascular diagnoses of the patients on admission to the operating room. More than half of the patients operated on in our center presented malformations at the level of the large vessels, with the most frequent diagnosis being a patent ductus arteriosus (PDA) in its different varieties, followed by aortic arch defects, among which the interruption of the aortic arch and aortic coarctation stand out. In this last pathology, it is important to mention the concomitant presence of aortic arch hypoplasia. Atrial malformations were the second cause of surgical intervention, with ostium secundum type atrial septal defect (ASD) being the most frequent. All of them corresponded to those without margins for intervention, except for one case of failure due to this therapeutic modality. It is worth mentioning that there was another case of common atrium ASD with concomitant persistent left superior vena cava. Ventricular defects occurred in less than a quarter of the patients, with a perimembranous ventricular septal defect (VSD) predominance, except for two infundibular VSD cases. Although RACHS-1 level 1 predominates in this series, it should be noted that a significant percentage of the operated patients had a greater complexity, such as an unclassifiable level.
POSTOPERATIVE RESULTS
Table 3 shows the operative morbidity, which occurred in less than a quarter of the patients in our series. Complications include respiratory infections, cardiac arrhythmias, surgical wound dehiscence, and hemopneumothorax. Table 4 details the cause of mortality according to age group, underlying cardiovascular diagnosis for each deceased patient. It can be observed that more than half of the patients who died were neonates and infants (56%), most of whom had critical congenital heart disease. The most frequent causes of death were cardiogenic shock, septic shock, and pulmonary arterial hypertension. Table 5 shows the mortality rates of our study series compared to the RACHS-1 scale, where we can observe a statistically significant difference in those patients with category 4 only, who had higher mortality than predicted.
DISCUSSION
Congenital heart defects are the most common malformations at birth and include a wide range of pathologies and surgical techniques that are applied to a no less wide spectrum of patients. Currently, mortality from these diseases are considered as an indicator of progress. This was established at a global level in the sustainable development as a goal in order to reduce deaths in children under five years of age with these malformations. In Mexico, the health system was traditionally centralized in large cities, which promoted that patients with congenital heart defects received late and fragmented care, leading mortality from these diseases to be 1.3 times higher compared to international statistics.7 In the last two decades, the Ministry of Health implemented a regionalization care project for patients with congenital heart defects with the objective of diminishing mortality. The Cardiovascular Diseases Committee of the European Association of Cardiothoracic Surgery recommends that one hospital with cardiovascular surgery service must be established for every 4 million inhabitants.8 Taking into account these recommendations, and knowing the population of our country in 2010, at least 25 hospitals with these characteristics were required. Despite this fact, in that year there were only 11 hospitals in the country, of which 8 of them were in Mexico City. In order to improve the coverage of specialized care at a national level, High Specialty Regional Hospitals were built, among which our hospital center was created. Our work team was a pioneer in the 10-year cardiac surgical experience that is presented in this study starting in 2013. Therefore, the initial percentage of pediatric patients operated on was low.
This study presents the results of our surgical procedures performed by a single surgeon on patients under 18 years of age with congenital heart disease at a third level regional hospital. This historical experience in cardiovascular surgery at our site began with a 12-year-old boy with patent ductus arteriosus, which was closed without complications and was chosen particularly for the high success rate of the surgical procedure.
According to the literature, congenital heart defects mainly affect males. The data analysis of this study disagrees with this statement because a predominance of the female gender was observed in our series. However, it was also documented that atrial septal defect and patent ductus arteriosus were more frequent in women as observed in this study. In contrast, aortic stenosis, coarctation of the aorta and transposition of the great arteries were more frequent in males and were more serious heart diseases as well.9 The procedures in these series were performed in schoolchildren and preschoolers. This is observed since, at the beginning of the congenital heart disease surgery program in our institution, we chose patients of these ages because they had lower surgical risk.
In the last 10 years of our experience, the most frequent heart disease was patent ductus arteriosus, probably due to hypobaric hypoxia associated with the altitude of the State of Mexico, where most of our patients came from.10 The most frequently occurring heart diseases worldwide are ventricular septal defect, atrial septal defect, patent ductus arteriosus, pulmonary stenosis, tetralogy of Fallot, aortic stenosis, aortic coarctation, and transposition of the great arteries.11,12 The initial experience that we present in this article included a minority of complex heart diseases, due to the rigorous selection that we made in our patients. This strategy allowed us to obtain a favorable historical experience in a newly created hospital, with recent personnel, processes, and machinery. Additionally, the beginning of a surgical program with this type of cases also offered the advantages of a low failure rate, low probability of needing postoperative therapy, and decreased costs and complications.
When analyzing our results applying the mortality predicted by the RACHS-1 scale, we observed that in categories 1, 2 and 3 we had a mortality rate that was favorably compared with internationally accepted standards, despite being a newly created center (Figura 1). For this reason, we also observed a statistically significant increase in operative mortality with increasing complexity of the cardiovascular surgery performed. Although 90% of the surgical procedures presented were concentrated in the lowest risk groups (categories 1, 2 and 3), our surgical series was not exempt from higher risk procedures, as corroborated by the 9% of patients operated on with unclassifiable risk. It is important to highlight that this scoring system does not allow determining the operative mortality of individual patients since it does not incorporate other variables recognized as determinants of mortality, such as pathological background and the opportunity of surgery, among others.13,14 Although there is a direct correlation between surgical risk and RACHS-1 category, in our experience, patients with higher complexity were mainly associated with an incomplete or nonspecific diagnosis. It is also important to mention that the historical experience created was not only based on elective patients, because there were 22% of patients who required urgent intervention, which increased the complexity.
CONCLUSIONS
Developing a surgical program for congenital heart disease represents a great challenge for the health personnel involved, since a series of administrative, technical, economic and, mainly, human resources problems must be considered. Many of the patients who underwent surgery were candidates for therapeutic intervention. However, the appropriate specialists in this area were not available at the beginning of our center experience. Although this is a clear example that shows the need for strengthening of the cardiovascular team, it must also be recognized that its absence allowed the consolidation of our surgical service, since this therapeutic alternative is useful in these circumstances. Finally, this set the precedent for continuing to progress in the future for establishing our center as a regional reference in increasingly complex pathology in the area of congenital heart disease.
ACKNOWLEDGMENTS
Special thanks and recognition are extended to the whole team that participated in this pioneering experience of the congenital cardiovascular surgery program: Dr. Karla Felicia Martínez Medina, Dr. David Orozco Macías, Perf. Lic. Leticia Salado Lucero, Dr. José Luis Patiño Galeana, Dr. Miguel Sánchez Durán, Dr. Enrique Ramírez Netro (RIP), Dr. Miguel Ángel Chávez Martínez, Dr. Carolina Tortolero Sánchez, Dr. Hugo Cardoza Pacheco and Dr. Alma Rosa Sánchez Conejo.
REFERENCES
Juárez-García V, Camarillo-González FT, Rodríguez-Cornejo RE, Curi-Curi JP. Relationship between hypoxia and pulmonary microcirculation hemorheology in pediatric patients with patent ductus arteriosus operated at a moderate altitude. In Book: Hypoxia - Recent Advances in the Field of Hypoxic and Ischemic Tissue Damage. IntechOpen; 2023. doi: 10.5772/intechopen.1002882.
AFFILIATIONS
1 Department of Cardiovascular Surgery for Congenital Malformations, Regional High Specialty Hospital of Ixtapaluca. Ixtapaluca, State of Mexico, Mexico.
2 Faculty of Higher Studies Zaragoza, Autonomous National University of Mexico. Mexico City, Mexico
Funding: none.
Disclosures: the authors have no conflict of interest to disclose.
CORRESPONDENCE
Dr. Pedro José Curi Curi. E-mail: pcuricuri001@gmail.comReceived: 02-25-2025. Accepted: 04-21-2025.