|Year : 2018 | Volume
| Issue : 1 | Page : 50-56
Development of the taksande's score: A new scoring system for the diagnosis of pulmonary arterial hypertension
Amar M Taksande, Rewat Meshram, Amol Lohakare, Sadhana Purandare, Aakshi Gandhi
Department of Pediatrics, Jawaharlal Nehru Medical College, Wardha, Maharashtra, India
|Date of Web Publication||7-May-2018|
Dr. Amar M Taksande
Department of Pediatrics, Jawaharlal Nehru Medical College, Sawangi Meghe, Wardha - 442 102, Maharashtra
Source of Support: None, Conflict of Interest: None
Background: In the pediatric population, pulmonary arterial hypertension (PAH) is associated with a number of underlying diseases which causes significant morbidity and mortality. PAH in children is mainly idiopathic or associated with congenital heart disease (CHD). No scoring systems have been developed to aid in the diagnosis of PAH.
Aim: This study aims to develop a PAH scoring system that is more applicable to the children.
Materials and Methods: This prospective diagnostic study was conducted on the 428 CHD children admitted to a tertiary referral hospital. The pediatricians had examined independently and used the palpation and auscultation to detect the study participants for PAH (Index text). Echocardiography for PAH (Reference standard) was performed after the clinical assessments of the children. For the development of the new scoring system, the data collected which include anthropometry, clinical signs (tachypnea, cyanosis, pedal edema, and hepatomegaly) and the cardiovascular examination (Loud second heart sound, palpable P2, ejection click, dullness in 2nd intercostal space, and murmur). All statistical analyses were performed using STATA statistical software (version 10.0).
Results: The study population consisted of 428 children who had CHD. On examination, 71% children were wasted, 44.63% had tachypnea, 20% had cyanosis, 18.46% children had pedal edema, and 41% had hepatomegaly. Murmur was present in 53% of the study cases. The optimal cutoff threshold score derived from the receiver operating characteristic curve analysis was 5. Based on this optimal cutoff threshold, the calculated sensitivity and specificity were 85.5% (95% confidence interval [CI] 76.7%–91.8%) and 92.5% (95% CI 89.1%–95.1%), respectively. The positive predictive value and negative predictive value were 76.6% and 95.6%, respectively. The diagnostic accuracy of the new score was 89.71.
Conclusions: This new PAH scoring system is easy and simple to apply as the majority of the parameters can be obtained from a routine history and clinical examination.
Keywords: Children, echocardiography, heart lesion, pulmonary hypertension
|How to cite this article:|
Taksande AM, Meshram R, Lohakare A, Purandare S, Gandhi A. Development of the taksande's score: A new scoring system for the diagnosis of pulmonary arterial hypertension. Nig J Cardiol 2018;15:50-6
|How to cite this URL:|
Taksande AM, Meshram R, Lohakare A, Purandare S, Gandhi A. Development of the taksande's score: A new scoring system for the diagnosis of pulmonary arterial hypertension. Nig J Cardiol [serial online] 2018 [cited 2019 Jan 24];15:50-6. Available from: http://www.nigjcardiol.org/text.asp?2018/15/1/50/231971
| Introduction|| |
Pulmonary hypertension (PH) has been an increasingly recognized in clinical practice over the last 25 years. PH is a serious and unrelenting pulmonary vascular disorder in children that significantly decreases their lifespan and affects the functional quality. This mainly deals with the World Health Organization (WHO) Group 1 pulmonary hypertension which is designated pulmonary arterial hypertension (PAH). In the pediatric population, it is associated with a number of underlying diseases which causes significant morbidity and mortality. PAH in children is mainly idiopathic or associated with congenital heart disease (CHD). In developed countries, the prevalence of PAH associated with systemic-to-pulmonary shunts has been estimated to 1.6–12.5 cases/million adults, with 25%–50% of this cases affected by the Eisenmenger's syndrome (ES).,, The higher proportions of patients with repaired or unrepaired systemic-to-pulmonary shunt are reported to have PAH in other forms than the ES., There is no specific data are available on the prevalence and incidence of PAH associated with systemic-to-pulmonary shunt in children. If PAH diagnosed early, a better quality of life can be provided with the new therapeutic options. The diagnosis of PAH is increasing because of increased clinicians awareness and the routine use of Doppler echocardiography. The diagnosis of PAH is based on clinical history and examination combined with echocardiography. Despite being a common problem, PAH remains a difficult diagnosis to establish, particularly among the infant and children. The diagnostic accuracy can be improved through the use of echocardiography or computed tomography imaging. Nevertheless, these modalities are very costly and may not be readily available when they are required. Making arrangements for these diagnostic modalities may lead to further delays in diagnosis and surgery. No scoring systems have been developed to aid in the diagnosis of PAH. Thus, the objective of this study was to develop a PAH scoring system that is more applicable to the children.
| Materials and Methods|| |
Study population and study design
This prospective diagnostic study was conducted on the 428 CHD children admitted to a tertiary referral hospital (Pediatric Department AVBRH, Sawangi Meghe, Wardha), between September 2012 and 2016.
Inclusion criteria were children with clinically suspected congenital heart disease. A written informed consent was obtained from one of the parents of the children for inclusion in the study.
Exclusion criteria were critically ill patients and severe anemia (blood hemoglobin [Hb] level ≤6 g/dl) were excluded from the study.
The study was approved by the Research Ethics Committee of the Datta Meghe Institute of Medical Sciences, Jawaharlal Nehru Medical College, Sawangi Meghe.
Data and specimen collection
The medical personnel were educated and trained about the PAH at the beginning of the study to assure the consistency and avoid interpersonal biases of calculating the proposed scores. The pediatrician was blind to the details of history, general examination, and laboratory investigation findings of the study participant. They had been examined independently and used the palpation and auscultation to detect the study participants for PAH (Index text). To access reliability of PAH, other pediatrician was evaluated each child independently and blinded and the interval between the observer's examinations ranged between 60 and 90 min. The data collected were child age, gender, the presenting symptoms, anthropometry, and clinical signs (tachypnea, cyanosis, pedal edema, and hepatomegaly). The cardiovascular finding noted were loud second heart sound, palpable P2, ejection click, dullness in 2nd intercostal space (ICS), and murmur.
Echocardiography for PAH (Reference standard) was performed after the clinical assessments of the children before starting treatment. All the study participant was undergone an echocardiographic assessment of the PAH on the same day by cardiologist, who was unaware of the clinical condition and the observations made by the clinician, using an echocardiography machine (GE vivid E) Color Doppler system with a multifrequency 3.5-5 MHz probe. Appropriate transthoracic transducer selection was made at the time of the evaluation. Cardiac measurements were performed according to the guidelines of American Society of Echocardiography. PH is defined as a peak tricuspid regurgitation velocity (TRV) of at least 2.5 m/second equating to a pulmonary artery pressure (PAP) of at least 30 mmHg. Mild pressure half-time (PHT) is defined as peak TRV of 2.5–2.9 m/s, corresponding to PA systolic pressure of 30–40 mm Hg. Moderate PHT means PAP between 41 and 55 mmHg and severe PHT means PAP higher than 55 mmHg. Patients with no measurable TRV or TRV ≤2.5 m/s will be considered to have normal PA pressures.,
The following parameter was used for screening the child for PAH.
According to the WHO, wasting is defined as weight for height <–2 standard deviation of the WHO Child Growth Standards median.
Cyanosis, a bluish-purple discoloration of the tissues due to an increased concentration of deoxygenated Hb in the capillary bed, results from a variety of conditions, many of which are life-threatening. Central cyanosis is evident when the systemic arterial concentration of deoxygenated Hb in the blood exceeds 5 g/dL (3.1 mmol L) (oxygen saturation ≤85%).
Accumulation of excessive amount of fluid in the extravascular interstitial space of the body. Pedal edema is one of the sign of right-sided heart failure. Firm pressure on the pretibial region for 10–15 s may be necessary for detection of edema in less severe disease.
The presence of a palpable liver does not always indicate hepatomegaly. In general, a liver edge >3.5 cm in newborns and >2 cm in children below the right costal margin suggests enlargement.
Tachypnea/fast breathing in children
Tachypnea is often used as a clinical marker for pneumonia in patients of all ages. For children, the WHO recommends age-specific definitions of tachypnea: age <2 months, respiratory rate (RR) ≥60 breaths per minute (bpm); 2–12 months, RR ≥50 bpm; and 1–5 years, RR ≥40 bpm.
Heart murmurs are sounds other than the normal heart sounds emanating from the heart region. Heart murmurs are most frequently categorized by timing, into systolic heart murmurs and diastolic heart murmurs, differing in the part of the heartbeat on which they can be heard.
Palpable second heart Sound (P2)
Palpable second heart sound in the pulmonary area means palpable P2, secondary to PAH.
The second heart sound is frequently accentuated in patients with pulmonary hypertension. This is because the intensity of P2 is dependent on the velocity of blood coursing back toward the right ventricle after ventricular contraction and the suddenness in which that motion is arrested by the closing valve. In patients with PAH, the diastolic pressure within the PA is high and therefore the velocity of blood moving toward the tricuspid valve is increased, resulting in an accentuated P2. Loud P2 is diagnosed, if it is heard at apex (normal P2 is not heard in apex) and if P2component is louder than A2 component in pulmonary area.
An ejection click (or ejection sound) follows the S1 very closely and occurs at the time of the ventricular ejection's onset. Therefore, it sounds like a splitting of the S1. However, it is usually audible at the base (either side of the upper sternal border), whereas the split S1 is usually audible at the lower left sternal border (exception with an aortic click). The pulmonary click is heard at the second and third left intercostals spaces and changes in intensity with respiration, being louder on expiration.
Dullness 2nd intercostals space
Percussion of the 2nd left intercostals spaces from midclavicular line to the sternum is resonant. Dullness of the same area could be due to dilated PA as in, pulmonary hypertension, ventricular septal defect (VSD), or patent ductus arteriosus (PDA).
For the development of new scoring system, the data collected which include anthropometry, clinical signs (tachypnea, cyanosis, pedal edema, and hepatomegaly) and the cardiovascular examination (loud second heart sound, palpable P2, ejection click, dullness in 2nd ICS, and murmur). The inclusion of these ten parameters was agreed on by a panel of pediatrician at AVBRH Hospital. These ten parameters form the basis of the new PAH scoring system. The probability of each parameter was calculated and scores of 0.5, 1.0, or 2.0 points were allocated to each parameter based on its probability, with extra weightage provided to three clinical signs: loud second heart sound, palpable P2, and dullness in 2nd ICS. Confirmation of PAH as the final diagnosis was obtained from the transthoracic echocardiography.
All statistical analyses were performed using STATA 10.1 version, (StataCorp, 4905 Lakeway Drive, College Station, Statistics/Data Analysis, Texas, USA). The binomial data were analyzed using a nonparametric Chi-square test. The probability and odds ratio (OR) for each parameter were derived using logistic regression analysis. The receiver operating characteristic (ROC) curve at the optimal cutoff threshold score for the new PAH scoring system was derived using the STATA statistical software. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) at the optimal cutoff threshold score were also derived from the ROC. The sample size was calculated for a power of 80% and an alpha error of 0.05. Estimated ORs with 95% confidence intervals (CIs) and P < 0.05 were used to evaluate the statistical significance of the associations and correlations between variables. The Taksande's scores, as assessed by residents, were cross-tabulated against the Taksande's scores as assessed by consultants. For each cross-tabulation, interrater agreement was assessed using the weighted kappa statistic. A kappa value of 0 indicates that the observed agreement is completely due to chance, whereas a value of 1 indicates a perfect agreement. Other values were graded according to the following guidelines: Kappa scores were interpreted as follows: poor 0.01–0.20, moderate 0.21–0.40, fair 0.41–0.60, good 0.61–0.80, or excellent 0.81–1.0
| Results|| |
The study flow chart is shown in [Figure 1]. The study population consisted of 428 children who had congenital heart disease. The mean age of the children was 4.79 ± 0.20 years, with a male-to-female ratio of 1.4:1. Characteristics and pattern of CHD in cases are shown in [Table 1]. The mean weight of the study cases was 13.09 ± 0.42 kg. A final diagnosis of PAH was confirmed on echocardiography in 96 (22.43%) cases, while 332 (77.57%) children had no evidence of pulmonary hypertension. The parameters included in the new Taksande's scoring system consisted of wasting, tachypnoea, cyanosis, pedal edema, hepatomegaly, palpable P2, dullness in the 2nd ICS, loud P2, ejection click, and murmur. The probabilities for PAH were calculated for each of the ten parameters, as shown in [Table 1]. Scoring of the parameters was done based on the probability of PAH.
|Table 1: Characteristics and pattern of congenital heart disease in cases|
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The presence of pedal edema and hepatomegaly is the sign of right heart failure, hence they were combined or a score of 1.0 point; thus, a score of 0.5 point was allocated to each of these parameters. The signs of palpable P2, dullness in the 2nd ICS, and loud P2 were weighted highly by our panel of local pediatrician and cardiologist, as the presence of these three clinical signs was highly indicative of PAH. Hence, these three parameters were scored with 2.0 points each. The other parameters (tachypnea, ejection click, and murmur) were all scored with 1.0 point each [Table 2].
|Table 2: The probability of pulmonary arterial hypertension for each parameter, with the scoring of parameters based on probabilities and extra weightage|
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On examination, 71% children were wasted, 44.63% had tachypnea, 20% had cyanosis, 18.46% children had pedal edema, and 41% had hepatomegaly. Murmur was present in 53% of the study cases. The optimal cutoff threshold score derived from the ROC analysis was 5, as shown in [Figure 2]. Based on this optimal cutoff threshold, the calculated sensitivity and specificity were 85.5% (95% CI 76.7%–91.8%) and 92.5% (95% CI 89.1%–95.1%), respectively. The PPV and NPV were 76.6% and 95.6%, respectively. The diagnostic accuracy of the new score was 89.71. All possible cut points, with their associated sensitivity and specificity, are also shown in [Table 3]. Interrater reliability of Taksande's score between pediatric residents and consultant pediatricians was determined for 428 cases. The total Taksande's scores showed excellent agreement between residents and consultants, as confirmed by the weighted kappa coefficient of 0.7484.
|Figure 2: The optimal cutoff threshold score derived from the receiver operating characteristic analysis|
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|Table 3: Cut points for diagnosing pulmonary arterial hypertension in all possible scores|
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| Discussion|| |
PH is a complex problem characterized by nonspecific signs and symptoms and having multiple potential causes. All patients of CHD should be examined for PAH. The assessment of CHD patients is that of recognizing residual and progressive hemodynamic cardiac lesions that can cause functional deterioration and influence on the quality of life. PAH is usually the result of a large unrepaired VSD or a large PDA or late repair of a posttricuspid defect or rarely may indeed develop later on in life, even after timely repair of a defect, or even in the absence of significant residual hemodynamic lesion. It significantly affects exercise capacity, and thus, the quality of life get affected. In addition, strenuous or extreme work can be dangerous and should be discouraged in PAH-CHD patients. Children with large pulmonary blood flow (PBF) and elevated pressures generally do not develop irreversible pulmonary vascular changes before 1–2 years of age, while children with truncus arteriosus can develop pulmonary vascular lesions in infancy. PAH is difficult to diagnose early because it is not often detected in a routine physical exam. Even when the condition is more advanced, its signs and symptoms are similar to those of other heart and lung conditions. Pediatric echocardiography is a noninvasive tool to evaluate heart function and structure in children. Because of chronic right ventricular (RV) pressure overload, most children present with enlarged right-side chambers, RV hypertrophy, increased interventricular septal thickness, and reduced global RV systolic function. Pulmonic and tricuspid insufficiencies are detected with Doppler interrogation., In the cardiac catheterization laboratory, the normal PA systolic pressure of children and adults is ≤30 mm Hg and the mean PA pressure is ≤25 mmHg at sea level. When the mean PA pressure is >25 mm Hg in a resting individual at sea level, then the diagnosis of PH can be made. The prevalence of advanced PAH in children and adults with CHD is 1.2% and 4.2%, respectively, in a tertiary center of São Paulo, Brazil.
PAH associated with large shunt lesions, such as VSD and PDA, is called hyperkinetic pulmonary hypertension. It is the result of an increase in PBF, a direct transmission of the systemic pressure to the PA, and an increase in pulmonary vascular resistance (PVR) by compensatory pulmonary vasoconstriction. If no vasoconstriction occurs, the increase in PBF is much larger and an intractable congestive heart failure results. Hyperkinetic pulmonary hypertension is usually reversible if the cause is eliminated before permanent changes occur in the pulmonary arterioles. The study conducted by Zhang et al. reported that the WHO Class I PAH accounted for 91.1%, CHD-PAH 67.3%, idiopathic pulmonary hypertension (IPAH) 23.8%, and other 8.9%. For patients with IPAH, the median time period between onset of symptoms and diagnosis by right heart catheterization was 38 months. Bobhate et al. found idiopathic or heritable PAH (29%), PAH associated with CHD (52%), left heart disease (5%), and lung disease (14%). Mean PAP was 43 ± 19 mmHg; mean PVRI was 9.7 ± 6 Wood units m2. Three percent of children with PH undergoing cardiac catheterization suffered adverse events. The histological feature of pulmonary vascular lesions in CHD consists of a sequence of lesions beginning with medial hypertrophy, followed by cellular proliferation and concentric laminar intimal fibrosis. Other changes like dilation lesions, fibrinoid necrosis, and plexiform lesions developed after reversal of shunt.
The clinical manifestations of PAH are mostly related to the degree of PA pressure elevation and the status of the right ventricle. The signs and symptoms of PAH in its early stages might not be noticeable for months or even years. As the disease progresses, symptoms become worse. Dyspnea and fatigue are the earliest and most frequent complaints. Headache syncope or chest pain also occurs on exertion, which generally represents more advanced disease. History of a cardiac defect or CCF in infancy is present in most cases of ES. Pediatric patients present with better preserved functional class, and parents should pay high attention to any children with unexplained shortness of breath, fatigue or syncope, as symptoms of PAH in children is often misleading. The patient with PAH associated with CHD (APAH-CHD) presents with unique challenges consisting of not only pulmonary vascular disease but also the complexity of the cardiac lesion. ES represents the severe end of the spectrum for disease in APAH-CHD. Over time, systemic-to-pulmonary shunting through cardiac defects increases PVR to levels significant enough to reverse shunting across the defect. Right heart catheterization is necessary for diagnosis.,,
No scoring systems have been introduced till date to help with the clinical decision-making process in achieving an accurate diagnosis of PAH in the fastest and cheapest way. However, only echocardiographic scoring systems for PAH was created by Kawataki. This new Taksande's PAH scoring system includes the ten parameters mentioned above in the material and methods. The minimum and maximum total scores achievable with this new Taksande's scoring system were 2 and 11, respectively. Based on this optimal cutoff threshold, the calculated sensitivity and specificity were 85.5% (95% CI 76.7%–91.8%) and 92.5% (95% CI 89.1%–95.1%), respectively. The PPV and NPV were 76.6% and 95.6%, respectively. Our results have shown that the clinical diagnosis of PAH can be facilitated by a Taksande's score. The score showed excellent agreement between residents and consultants (weighted κ = 0.7484.), and appeared to be accurate in predicting infants with PAH. The diagnostic accuracy of the new score was 89.71. This new PAH scoring system was specifically developed for our local patient group, but it is likely to be applicable to other developing and underdeveloped countries of the world. This new PAH scoring system is easy and simple to apply as the majority of the parameters can be obtained from a routine history and clinical examination.
| Conclusions|| |
The new PAH scoring system described in this study and referred to as the TAP (Taksande's PAH) score, in short, is promising and has good sensitivity, specificity, and diagnostic accuracy. TAP score is simple and easy to use and has been specifically developed for CHD children for diagnosing PAH.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]