Clinical-Epidemiological Profile of Congenital And Acquired Heart Diseases Admitted To A Private Hospital

Introduction

Congenital heart diseases (CHDs) are defined as abnormalities in the cardiocirculatory structure or function that are present since birth and can have a significant functional impact on the life of the individual [1]. They represent the majority of all mal- formations in live-born babies and are the most common cause (30% to 40%) of mortality among birth defects [2].

The prevalence of CHDs in the world population is uncertain due to the lack of records in many countries, but it ranged from 5 to 8 per 1,000 live births before the introduction of Doppler echocardiography and 8 to 12 after the widespread use of this diagnostic method [3, 1]. In Brazil, the presentation of CHDs is similar to those observed in other nations, generating an alert that the health system should be prepared to diagnose and treat these patients as early as possible to reduce costs, prevent possi- ble sequelae, and mitigate the emotional distress of the patients and their families [4]. In addition, due to substantial advances in the management of CHD, including improvements in surgical techniques, intensive support, pharmacotherapy, and early de- tection, treatment and results have improved drastically in recent decades [3, 5]. Thus, many of these new-borns will survive and thus increase the population of adults with CHD, which reinforc- es the importance of understanding the epidemiology of these diseases in order to support valuable changes in health policies and clinical practices [6].

Recognizing the profile of this population is essential because CHDs are among the main causes of neonatal morbidity and mortality, and their prevalence in the population is growing; however, cases are underreported and few studies on them have been published in Brazil, which reinforces the importance of producing epidemiological studies [6-2, 4]. The compilation of relevant information on the characteristics of children with CHD can reveal indicators related to the prevalence of cases, good care practices, and specific outcomes, contributing to the improvement of health services [4, 5].

In this context, the objective of this study was to understand the case series of congenital heart disease patients monitored in a private hospital of São Paulo and to report the clinical profile of these patients. These records will serve as a basis for future stud- ies and will allow for national and international collaborations.

Methods

This was a retrospective, longitudinal, observational interfer- ence study with a quantitative design that was conducted at private hospital and philanthropic in the city of São Paulo that has neonatal intensive care unit, paediatric intensive care unit, and paediatric ward beds. It has a monthly average of 4.6 ad- missions/month for treatment related to paediatric heart diseas- es (congenital, acquired, and arrhythmias). The patients were selected through the electronic medical records database, the TASY System, through the analysis of medical records of hospi- talizations from January 2016 to March 2020.

The cardiac paediatric group of the of this private hospital se- lected 502 patients according to the following inclusion criteria: patients with congenital heart disease, acquired heart disease, or arrhythmias. Data were collected through the use of an in- strument that covered clinical, sociodemographic, preoperative, and postoperative variables. It was formulated in RED CAP soft- ware, a clinical research management platform that is designed to create databases and that meets all regulatory requirements related to information security.

The patients were coded according to the international classifi- cation of diseases (ICD-10) and the International Pediatric and Congenital Cardiac Code (IPCCC). The main diagnosis was de- fined as the most complex congenital cardiac injury.

After collection, we organized the data in Excel spreadsheets, in which we ran descriptive analyses of the absolute and relative frequencies, represented by tables and graphs. This study was approved by the ethics committee, which waived the need for informed consent.

Results

In total, 502 patients were treated by the cardiac paediatric group from January 2016 to March 2020. Of these, 86.5% had congen- ital heart defects, 7.7% had acquired heart defects, and 5.8% had arrhythmias. A total of 50.2% were female, 49.6% were male, and 0.2% had an unrecorded sex.

Most of the patients admitted to hospital were admitted for elec- tive surgery, followed by transfer from an external hospital and being born in our obstetric centre.

Approximately 34.7% had related comorbidities. Seizures, pro- tein-calorie malnutrition, and encephalopathies were the main symptoms found. Syndromes were diagnosed in 126 patients, while 31 were under diagnostic investigation. Down syndrome was the most common, representing 58% of the syndromes (Ta- ble 1).

                                                                    Table 1: Presence of Syndromes

Regarding prematurity, 97 patients were born at less than 37 weeks, representing 19.3% of the study patients. Table 2 shows the distribution between extremely preterm, very preterm, moderate preterm, and late preterm infants [7].

Table 2: Prematurity in weeks

The distribution of the types of congenital heart disease, ac- quired heart disease, and arrhythmias are described in Table 3. The most frequent cyanogenic heart diseases found were tetral- ogy of Fallot (10.56%), transposition of the great arteries (6.38), hypoplasia syndrome of the left heart (5.98%), truncus arterio- sus (2.59%), right ventricular outflow tract double (2.99%), and pulmonary atresia with interventricular communication (4.18%). The most frequent acyanogenic heart diseases were total atrio- ventricular septal defect (10.56%), interventricular communica- tion (9.96%), aortic coarctation (9.56%), interatrial communica- tion (8.36%), and persistence of the ductus arteriosus (6.57%).

                                                                     Table 3: Distribution of Heart Diseases

The distribution of the type of cardiac physiology showed that 81.1% of patients had biventricular physiology and 18.9% had univentricular physiology. Among the patients treated by the cardiac paediatric team, 445 (88.65%) underwent some type of invasive intervention, such as surgery, haemodynamic procedure, or hybrid procedure. The need for combined interventions was observed in several cases. In total, 530 procedures were performed, of which 77% were surgeries, 18.5% were haemodynamic procedures, and 4.5% were hybrid procedures. Categorizing the surgical procedures by the adjusted risk for sur- gery in congenital heart disease (RACHS) score [8], we found a predominance of category 3 (38.4%), followed by category 2 (28.6%), category 1 (18.2%), category 4 (9.6%), and category 6 (5.2%). Most of the patients subjected to invasive procedures were neo- nates (31%) or infants (45.8%). Table 4 shows the age distribu- tion at the intervention date.

                                                               Table 4: Age of the patient at the invasive intervention

Discussion

The global prevalence of CHDs shows an increase of 10% every 5 years, which is due to the expansion of the use of echocar- diography and the improvement of technologies, according to a meta-analysis on the epidemiological situation of congenital heart diseases [9].

In the study hospital, 502 patients were referred by the cardio- paediatrics group, most often coming to us for elective surgery, but many also were transferred from other hospitals or were born in the obstetric centre of our hospital. In Brazil, a study on the local epidemiological situation estimated 25, 757 new cases of CHD/year, without considering the large amount of underre- porting, serving as a warning about the growing number of new cases and the need to direct investments to the care of this local population [10].

We saw no predominance of one sex over another, in line with several other studies [11, 12]; however, there are studies that re- port significant sex-related differences in specific heart diseases [13]. Some 19.3% of our sample were born prematurely. This high number is in line with the finding that 16% of children born with cardiovascular malformations are premature, according to a large English study [14]. In addition, an important issue in this population is the high mortality rate [14]. Premature infants who have CHD have a worse performance than those born at term because they are more vulnerable, and this condition remains a clinical and surgical challenge [15].

The most frequent CHDs found in the present study were acy- anogenic, which is in agreement with the epidemiological data from national publications [4-10, 16]. In international data, there is also a predominance of acyanogenic heart diseases [9, 17]. The prevalence of mild injuries is due to the greater use of echo- cardiography worldwide, which has increased the diagnosis rate. In addition, abortion in complex heart diseases is an option in many countries, which also influences the reduction of the inci- dence of these specific CHDs [9, 17].

Total atrioventricular septal defect (10.56%), interventricular communication (9.96%), and aortic coarctation (9.56%) are the most prevalent acyanogenic CHDs. Of the congenital cyanogen- ic heart diseases, the most commonly found were tetralogy of Fallot (10.56%), transposition of the great arteries (6.38), and left heart hypoplasia syndrome (5.98%). Other studies have found that tetralogy of Fallot was the most frequent cyanogenic CHD [9, 11]. Total atrioventricular septal defect was the most common acyanogenic CHD, but it is not the most prevalent in the world literature, which can be explained by the significant number of patients with Down syndrome (n = 91) in our study population [16].

In CHDs, the anatomy determines the cardiac physiology, and patients with univentricular physiology have a more complex clinical management and undergo numerous invasive interven- tions throughout life [18]. In this study, the classification of pa- tients according to physiology showed a prevalence of 81.1% with biventricular physiology, whereas univentricular patients represented 18.9% of the sample. This significant proportion presents a challenge for the entire multidisciplinary intensive care team. The physiology of a single ventricle may result from a series of anatomical lesions that are associated with a variety of physiological manifestations, requiring great expertise from the professionals involved in conducting treatment and surgical correction [18].

Syndromes were diagnosed in 31.2% of our sample, which is in agreement with the range found in the literature of 25 to 30% 19,20. Chromosomal diseases stood out, especially Down syndrome, representing 58% of the syndromes found. The high prevalence of chromosomal diseases corroborates the recom- mendation of the need to perform chromosomal studies in new- borns with multiple anomalies [4].

A total of 34.7% of our sample had associated comorbidities. The abnormal circulatory physiology in children with CHD in- fluences the development and functioning of other systems, with a significant impact on the clinical management and outcome of cases [21]. Seizures, protein-calorie malnutrition, and encepha- lopathies were the main comorbidities found in this study.

Syndromes, extracardiac anomalies, prematurity, low birth weight, and other associated comorbidities aggravate the chal- lenges associated with the treatment of CHD and are significant risk factors that affect the survival of these patients [21, 22]. The vast majority of patients included in the study (88.65%) underwent invasive intervention. In total, 530 procedures were performed, of which 77% were surgeries, 18.5% were haemo- dynamic procedures, and 4.5% were hybrid procedures. A risk score called RACHS-1 was used to categorize the surgeries. Despite some shortcomings, such as the low individual predic- tive power and the inability to classify all cardiac procedures, RACHS-1 is a well-used and widespread tool that categorizes CHD according to the expected mortality in six categories. Each of them is used as a predictor of postoperative mortality [23, 24]. In the present study, there was a predominance of category 3 (38.4%), followed by category 2 (28.6%), category 1 (18.2%), category 4 (9.6%), and category 6 (5, 2%). According to a Bra- zilian study of mortality in CHD using RACHS-1 in a single cen- tre, the mortality rate of category 3, the main one in this study, was 8.5%, though it ranged from 10.4% to 60% in internation- al reports [23]. On top of the high risk scores for the surgeries themselves, surgical complexity and treatment outcome are also strongly influenced by comorbidities and associated syndromes, both present in one-third of our sample [24]. This information reflects the need for highly specialized care, presenting a chal- lenge for clinical and surgical teams.

This study’s main limitation was its retrospective nature. Be- cause of this and the changes in the hospital evolution system,the data were limited.

Conclusion

This study comprehensively illustrates the epidemiological characteristics of a private hospital that also does philanthropic care. The complexity of the profile of the CHDs revealed that the integrated approach calling on professionals of various spe- cialties for the treatment of these patients is essential for a suc- cessful outcome.

Efforts must be made so that the team responsible for the care of complex CHD patients is highly specialized, as they have nu- merous associated comorbidities and syndromes and need spe- cific, high-level care. Organizing an intensive care unit focussed on paediatric cardiac care in hospitals that have a demand for it is a strategy to achieve high specialization of care and continu- ous improvements focussed on this population.

The issues raised are important because they serve as the basis of national comparative studies, which are scarce in the litera- ture, and because they explain the clinical profile of CHD treated in the private sector, which is erroneously thought to absorb less complex cases. Thus, the importance of each service knowing the particularities of the CHD population served is of paramount importance to perform investment management for quality care.

Acknowledgement

Gustavo Foronda. Pediatric Cardiologist: Study coordinator, critical revising of the work, substantial contributions to the de- sign of the work and final approval of the version to be pub- lished.

Vanessa F A de Melo. Cardiac: Research assistant drafting the work and final approval of the version to be published. Samia Medeiros Barbar: Analysis and interpretation of data of the work and final approval of the version to be published. Renata Fogarolli: Analysis and interpretation of data of the work and final approval of the version to be published. Carlos Eduardo Tossuniam: Critical revising of the work and fi- nal approval of the version to be published. Santiago Raúl Arrieta: Critical revising of the work and final ap- proval of the version to be published.

Statements and Declarations

The authors have no relevant financial or non-financial interests to disclose.

Ethics Approval

This study was carried out in accordance with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of the Municipal Secretary of Health of São Paulo. Ethics approval number: 25442019.3.0000.0086.

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