179 ORIGINAL ARTICLE Th e Pre-operative Assessment of Acyanotic Pediatric Patients Presented with and Role of Surgry in congenital heart diseases, A retrospective analysis Dhafer O Alqahtani, Ali A. Alakfash, Omar R .Altamim

Abstract Objectives: Th e aim of this study is to evaluate the incidence of congenital heart disease in patients referred solely because of heart murmur in pediatric age group and to assess the rule of medical and surgical management in patient with heart defects. Study design: It is retrospective analysis of all paediatric cases who presented with cardiac murmur. Materials and Methods:A retrospective database and echocardiographic review. All patients referred to King Abdulaziz Cardiac Center (KA CC) Riyadh, Kingdom of Saudi Arabia dur- ing the period from July 2007 till March 2009 for cardiovascular evaluation because of heart murmur detected during routine physical exam. We included any pediatric patient from the neonatal period till 14 years of age who had echocardiography in our center. Any patient with , those with diff erence in the blood pressure between the upper limbs and lower limbs of more than 15 mmHg, preterm neonates, any acquired heart disease and syndromic and critically ill patients were excluded from the study. Results: A total of 245 patients met the inclusion criteria. Median age and weight is 7 months (one day – 12 years), 7.85 Kg (1.9 – 54 Kg) respectively. Normal echocardiography was pres- ent in 163 patients (66.5%). Th e most encountered anomaly found was patent ductus arte- riosious (PDA) which was diagnosed in 27 patients (11 %) followed by (ASD) secundum in 26 patients (10.6%), then the VSD in 22 patients (9%), atrio-ventricular septal defect (AVSD) in 1 patient (0.4%), Coarctation of Aorta in 3 patients (1.2%), Tortuous of arch in 1 patients (0.4%), Pulmonary stenosis in 10 patients (4%), Mitral valve prolapse in 4 patients (1.6%) and the false tendon in 6 patients (2.4 %). Th e intervention by cardiac

King Abdullah Hospital, catheterization was done for 10 patients (4%). Surgical repair done in fi ve patients (2%). Th e Bisha, Kingdom of Saudi spontaneous closure was observed in 31 patients (12.7%). Th ere was 7 patients (2.9%) missed Arabia follow up. DO Alqahtani Conclusions: Th e prevalence of congenital heart disease in acyanotic pediatric patients re- King Abdulaziz Cardiac ferred with Heart murmur is signifi cantly high in Saudi population. Center (KA CC) Riyadh, Kingdom of Saudi Arabia Key words: Acynotic heart disease, patent ductus arteriosious, atrial septal defect, ventricular AA Alakfash OR Altamim septal defect, mitral valve prolapse, coarctation of aorta.

Corresopndence: Introduction: ological murmur. Sometimes the distinction is Dr Dhafer O Alqahtani, Consultant Paediatric Heart murmur is an abnormal Sound heard dur- not easy and the patient will be referred for evalu- Cardiologist, ing cardiovascular system( CVS) exam. It is a ation by a pediatric cardiologist, and very oft en an King Abdullah Hospital, Bisha, Kingdom of Saudi common fi nding in neonates and older children. echocardiographic exam will be requested, and Arabia Innocent murmurs are found in about 80% of some of general hospital no pediatric cardiologist Cell: 00966 504484494 normal hearts in pediatric age group. Th e treating or no near cardiac centre to them and need to de- email: drdhafer@hotmail. com physician should decide is it an innocent or path- cide is it pathological murmur need to be seen ur-

Pak J Surg 2015; 31(3): 180 gently by cardiologist or reassure the parents, it is caused by the shunt at the atrial level. Because innocent murmur or minor congenital heart dis- the pressure gradient between the atria is so ease need to seen on regular follow up to release small and the shunt occurs throughout the cardi- their stress from the result of CVS exam. ac cycle, in both systole and diastole, the left -to- right shunt is silent. Th e heart murmur in ASD Cardiac murmurs are audible turbulent sound originates from the pulmonary valve because of waves through crossing the valves or shunts le- the increased blood fl ow passing through this sion emanating from the heart and vascular normal-sized valve, producing a relative stenosis system. in the range of 20 to 20,000 Hz. Heart of the pulmonary valve and this will not happen murmurs are common in neonates, infants, and before age of six months. Th e VSD murmur de- children. Where as only 0.8% to 1% of the popu- termined by the size and the level of pulmonary lation has structural congenital cardiac disease, vascular resistance (PVR). With a small defect as many as 80% of the population has a heart and lowPVR harsh murmur are audible and this murmur sometime during childhood. Most will not happen until the infant reaches 4 to 8 heart murmurs are innocent, and they must be weeks of age . Th e common congenital obstruc- distinguished from pathologic murmurs of con- tive lesions to ventricular output are AS, PS, and genital or acquired cardiac diseases. Th ere are COA. All these obstructive lesions produce the two types of murmur, Innocent murmur and an ejection systolic murmur it will heard aft er pathological murmur. birth.

Th e innocent murmur or functional murmur During cardiac for murmur many arise from cardiovascular structure in absence factors aff ected the sound waves which have of anatomical abnormalities. All patient with three dimensions: intensity, frequency, and innocent murmur have no symptom or sign timbre or quality like thickness of chest wall, if of congestive , the intensity is less patient quit during exam, Size of diaphragm of than grade 3, normal ECG and cardiac size and if small will mask the soft systolic shadow in chest X-ray. If the murmur associated murmur, and the experience of the physician, with symptoms or signs of congestive heart and the hyper dynamic circulation. failure, strong family history of CHD, dysmor- phic feature, cyanosis, diastolic murmur, loud Materials and Methods: systolic murmur and thrill, if no sign of hyper- A retrospective database and echocardiographic dynamic ( anemia or fever ), abnormal or weak review. All patients referred to King Abdulaziz peripheral , or abnormal heart sound most Cardiac Center (KA CC) Riyadh, Kingdom of likely pathological murmur and need cardiology Saudi Arabia during the period from July 2007 consultation either urgently or routine follow up till March 2009 for cardiovascular evaluation according for the case situation. because of heart murmur detected during rou- tine physical examination. We included any pe- Th e causes of cardiac murmurs in pediatric diatric patient from the neonatal period till 14 group are related to the age of the patient at pre- years of age who had echocardiography in our sentation and the causes of congenital heart dis- center. Any patient with cyanosis, those with ease are varied and may include genetic (gene or diff erence in the blood pressure between the chromosomal) disorders, syndrome complexes, upper limbs and lower limbs of more than 15 metabolic disorders, or teratogenesis and the mmHg, preterm neonates, any acquired heart causes of acquired heart diseases in children in- disease and syndromic and critically ill patients clude rheumatic fever, endocarditis, and cardiac were excluded. injury caused by systemic illnesses. Results: Th e heart murmur with minor congenital heart A total of 245 patients met the inclusion crite- disease depend in the lesion like in ASD is not ria. Median age and weight is 7 months (one day

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30 genital heart disease which diagnosed by echo- cardiography. 25

20 In our study, the percentage of innocent murmur to have CHD is low ( 60 % )compare to other 15 study ( 80%). Th e loudness grade of the systolic 10 murmur holds key information, but grading the

5 loudness might be physician-dependent. Th e strong predictive power of the murmur grade 0 PDA ASD VSD AVSD Coarctation Tortuous Pulmonary Mitral False and the good reproducibility make it a reliable of aorta of arch stenosis valve tendon prolapsed tool in the bedside decision whether a murmur needs further evaluation. Figure 1: In our study the false tendon of the cordi (cordi – 12 years), 7.85 Kg (1.9 – 54 Kg) respectively. tendni)which connect between papillary mus- Normal echocardiography was present in 163 cle and intraventricular septum and one of the patients (66.5%). Th e most encounted anomaly known cause of ejection systolic murmur at left found was PDA which was diagnosed in 27 pa- upper border of chest. It is a variant of normal tients (11 %) followed by ASD secundum in 26 heart structure patients (10.6%), then the VSD in 22 patients (9%), AVSD in 1 patient (0.4%), Coarctation Th e ductus arteriosus is a fetal vascular connec- of Aorta in 3 patients (1.2%), Tortuous of arch tion between the main pulmonary artery and the in 1 patients (0.4%), Pulmonary stenosis in 10 aorta that diverts blood away from the pulmo- patients (4%), Mitral valve prolapse in 4 patients nary bed. Aft er birth the DA undergoes active (1.6%) and the false tendon in 6 patients (2.4 constriction and eventual obliteration. A patient %). Th e intervention by cardiac catheterization ductus arteriosus (PDA) occurs when the DA was done for 10 patients (4%). Surgical repair fails to completely close postnataly. Most com- done in fi ve patients (2%). eTh spontaneous monly the DA arises from the left innominate closure was 31 patients (12.7%). Th ere was 7pa- artery and inserts into the region of the proximal tients (2.9%) missed follow up. Th ere were 23 left pulmonary artery (1). patients (9.4%) still on follow up. Th e fetal DA is kept patent by low arterial oxy- Discussion: gen content and circulating prostaglandin E Th e Pediatrician and general physicians usually 2 (PGE ) which is produced in part by the pla- identify patients with murmur. these physicians 2 centa (2).Aft er delivery, circulating PGE levels referred most of the patients to be evaluated by 2 falls because of reduced production follow- pediatric cardiology either in urgent base or in ing removal of the placenta an increased PGE regular follow up to rule out cardiac lesion. 2 clearance due to increase in circulating level of In our study, show the innocent murmur was prostaglandin dehydrogeria (3) construction of 65% of referred cases and the acynotic cardiac DA usually result in functional haemodynamic lesion and need follow up was present in 35 % closure within 10-15 hours aft er delivery (4),(5), the patient who need intervention either surgi- closure begin at the pulmonary end of the DA cal or intervention catheterization was 6 % of and proceeds towards the aorta end (6). these lesions. Th e incidence of patient ductus arteriosus has In our study, we didn’t identifi ed the loudness increased dramatically over the last two decades. grade of the murmur, systolic or diastolic, and Th is is due to improved survival rate of prema- the presence of a pathologic ECG and chest x- ture infants because the incidence of PDA sig- ray which signifi cant positive predictors for con- nifi cantly increase infants born before 30 weeks

Pak J Surg 2015; 31(3): 182 gestation. Th e reported incidence of an isolated Ventricular septal deff ects: Twenty percent of all PDA among term infants range from .03 to patients with congenital heart disease have an .08 percent (7). I n a population based study of isolated ventricular septal defect (VSD). VSD 400,000 term infants born in Atlanta from 1998 also occurs in combination with other congeni- to 2005, the reported incidence of PDA was 2.9 tal heart defects, as in an atrioventricular canal, per 10,000 live births(8).Th ere is a female pre- tetralogy of Fallot, and transposition of the great dominance for PDA with a 2:1 female to male arteries (D-TGA) ration in most series of term infants. Th e inci- dence of PDA is also greater in infants born at Th e management of ventricular septal defects high altitude compared with those. (VSDs) varies depending upon several factors, including. Atrial septal defects (ASDs) are common, ac- counting for approximately 13 percent of con- Management of Ventricular Septal Defect: No genital heart disorders. Congenital defects of intervention is usually required for patients with the atrial septum are common, accounting for small defects. Th ese patients are typically as- approximately 13 percent of congenital heart ymptomatic and have a reasonable expectation disorders, with a reported birth prevalence of of spontaneous closure or decrease in the size of approximately 2 per 1000 live births 9. the defect over time. Medical therapy is required for patients with symptoms that are primary Atrial septal defects can be classifi cations into due to heart failure. For patients with moderate various types of atrial sept al defects (ASDs) are defects, medical management may adequately classifi ed on their diff erent anatomic locations address the needs of the patients. Surgical cor- and abnormal embryogenesis;Secundum ASD, rection may be indicated in patients who are not Primum ASD, Sinus venosus ASD, and Coro- adequately managed by medical therapy, and in nary sinus ASD those at risk for long term signifi cant sequelae (eg, irreversible pulmonary hypertension or val- Patient foramen ovale (PFO) is also an open var damage) that can be prevented by surgical communication between the right and left atria; repair. however, a PFO is not considered an ASD be- cause no septal tissue is missing. Dietary interventions may facilitate weight gain in infants with moderate to large ventricular sep- Secundum defects account for approximately 70 tal defects (VSDs). Th ese infants have increase percent of all ASDs, and occur twice as oft en in caloric needs due to an increased metabolic 11 females as in males . Familial recurrent rate has demand and may need a caloric intake that is 12 been estimated to be about 7 to 10 percent . A greater than 150 kcal/kg per day 14. comprehensive literature review reported a me- dian incidence of 564 per million live births11. Patients with advanced heart failure require sur- Th e Holt-Oram (heart-hand) syndrome is an gical repair. However, initial medical supportive autosomal dominant disorder characterized by measure may be needed in patients at the time upper limb defects (oft en deformities of the of diagnosis prior to surgical intervention, and radius, carpal bones, and/or thumbs) and car- include the following: diac septal defects, most commonly a secundum ASD 13. Administration of the angiotensin-converting enzyme (ACE) inhibitor to reduce the aft erload Th e primum type ASD develops if the septum resulting in improved heart failure symptoms. primum does not fuse with the endocardial Diuretics are the fi rst line of pharmacologic cushions, leaving a defect at the base of the in- therapy for infants who present with symptoms teratrial septum that is usually large. Th is type of heart failure (eg, and failure to of defect accounts for 15 to 20 percent of ASDs. thrive).Diuretic, inotropic agent, immunopro-

Pak J Surg 2015; 31(3): 183 phylaxis also place a role in medical manage- right ventricular out fl ow at the level of pulmo- ment. Indications for closure of VSD in infants nary valve. Th e obstruction can occur at several and young children include infants <6 months diff erent location, valvar stenosis is the most (,3 months for those with trisomy 21) who commonly type of PS and is characterized by have uncontrolled heart failure despite maxi- fused or absent commissures with thickened mal medical and dietary interventions or who leafl ets of the pulmonary valve. In most patients have pulmonary hypertension. Patients with a valve is a dome shaped structure with a small or- persistent signifi cant shunt (ratio of pulmonary cfi ce21. Dysplastic pulmonary valves are another to systematic blood fl ow, Qp:Qs.>2:1) and el- form of valvar PS, the valves are oft en irregular evated pulmonary artery (PA) pressures should with prominent leafl et thickening leading to a undergo surgical repair.Th e optimal manage- small hypoplastic valve annulus and reduced ment remains controversial for patients with mobility 22. persistent left ventricular (LV) dilation and or Qp:Qs shunt >2:1 with normal PA pressures15. Subvalvar PS is uncommon and is caused by Direct patch closure of the defect under cardio- primary fi bromuscular narrowing below the pulmonary bypass and/or deep hypothermia pulmonary valve is oft en associated with other is the single-stage procedure of choice in most congenital cardiac condition including double children and at most centers. Primary closure is chambered right ventricle and tetralogy of fallor 23. associated with an excellent outcome with low (TOF) rates of mortality and reoperation 16,7. Current Valvar PS with dysplastic pulmonary 24 valves is operative mortality for the single-stage direct associated with Noonan Syndrome. Although closure in most centers is less than 1 percent 16. there are some variability in the cardiac fi ndings Th e long term outcome for children who un- based on the severity of the defect. Th e features dergo surgical closure of VSD in childhood is which are distinctive for valvar P.S. is the fi rst generally excellent. Early surgical repair results normal heart sound which is typically followed in near normal long-term growth in the vast ma- by an audible click, the closer is the click to the jority of patients, and most survivors remain as- fi rst heart sound, the more severe is the P.S. until ymptomatic and lead normal lives 17,18. it merges with fi rst heart sound . eTh character- istics murmur of valvar P.S. is a systolic ejection Coarctation of the aorta is a narrowing of the de- murmur heart at the 2nd left intercostal space. scending aorta, which is typically located at the insertion of the ductus arteriosus just distal to In summary, the patient’s age, the loudness grade the left subclavian artery. Th is defect generally of the systolic murmur, and pathologic fi ndings results in left ventricular pressure overload. Co- in routine general exam, ECGs and chest x-rays arctation of the aorta accounts for 4 to 6 percent can guide clinicians toward a clinically reason- of all congenital heart defects with a reported able and cost-eff ective use of echocardiography. prevalence of about 4 per 10,000 live births7. It Pediatric patients with a systolic murmur graded occurs more commonly in males than in females ≤2/6 had innocent murmurs in 60%. (59 versus 41 percent). Conclusions: Although there are acquired causes, the etiology Careful initial clinical evaluation by General of coarctation of the aorta is generally congeni- physicians and Pediatricians including symp- tal. Reduced antegrade intrauterine blood fl ow toms and signs of congestive heart failure, any causing underdevelopment of the fetal aortic dysmorphic features, diff erent in blood pressure arch19. Migration or extension of ductal tissue between upper and lower limbs, Oxygen satu- into the wall of the fetal thoracic aorta20. ration, auscultation fi niding and abnormal ECG or CXR can help us to diff erentiate between an Pulmonary stenosis is defi ned as obstruction to acyanotic and non cyanotic congenital heart le-

Pak J Surg 2015; 31(3): 184 sions and who need to be evaluated by cardiolo- 12. Caputo S,Cappozzi G,et al Familial recurrence of congenital heart diseases with ostium secondum atrial septal defect. Eur gist in urgent assessment or regular follow up. Heart J 2005 26,2179. Careful follow-up will help us to identify these 13. Basson CT,Cowly GS et alTh e clinical and genetic spectrum of the Holt-Oram syndrome(heart and Hand syndrome) N cases which requires surgical intervention. Engi J Med 1994;330 385. 14. Rudolph WM. Vetricular Septal Defect in congenital diseases References: of the Heart Clinico-Physiological Consideration. Rudolph 1. Knight,, Edwards JER,Right Aortic Arch,Types and associated AM(ED),Futura Publishing Company, New York 2001 p197. Cardiac anomalies circulation1974;50;1047. 15. Gumbiner CH,takao A et al,Th e Science and Practice of Pedi- 2. Smith GC Th e Pharmacology of Ductus Arteriosus Pharmocol atric Cardiolgy 2 nd ed,Garson A Bricker JT et al William & Rev 1998 ;50;35 Wilkins, Baltimore 1998 p1119. 3. Loft inCD,Trivedi DB,Langenback R et al ;Cyclogenase 1- Se- 16. Bol-Raap G ,Weerhem J,et al Follow up aft er surgical closure of congenital ventricular septal defect,Eur J Cardiothorasic Surg lective inhibition prolongs gestation in mice without adverse 2003;24;511. eff ect on ductus arteriosus J Clin Invest 2002; 110549. 17. Meijboom F, Szatmari A, et al, Long term follow up aft er surgi- 4. Coggins KG, Latour A , et al , Metabolism of PGE2 by Prosta- cal closure oo ventricular septal defect in infancy and child- glandin dehydrogenase is essential for remodeling the Ductus hood, J Am Coll Cardiol 1994;24;1358. arteriosus. Nat Med 2002;8;91. 18. Danilowicz D,Presti S et al, Result of urgent or emrergency re- 5. Heymann MA,Rudolph AM et al Control of the ductus arte- pair for symptomatic infants under 1 year of age with single or riosus Physiol Rev 1975;55;62. multiple ventricular septal defect; Am J Cardiol 1992; 69;699. 6. Gitt enberger-de,Groot AC,et al Histologic studies on normal 19. Rudlf AM,Heymann MA et al ,Haemodynamic consider- and persistent ductus arteiosus in the Dog J am Coll Cardiol ation in the development of narrowing of aorta Am J Cardiol 1985;6;394. 1972;41 268. 7. Hoff man JI,Kaplan S eTh incidence of congenital heart dis- 20. Wielenga G,Dankmeijier et alCoarctation of Aorta J Pathol eases J Am Coll Cardiol 200239-1890. Bacteriol 1968 95 ;265. 8. Reller MD,Strickland MJ, et al Prevelence of Congenital 21. Edwards JE,Congenital malformations of the heart and great heart defects inmetropolitan Atlanta,1998-2005 J Pediatr vessels In; Pathology if the Heart, Gould SE,Chales C, Th om- 2008;153;807. as Publisher, Springfi eld, IL 1953. 9. Van der Linde D,Konings EE et al Birth prevalence of con- 22. Becu LSomerville J et al Isolated Pulmonary valve stenosis as genital heart diseases worldwide;a systemic review and meta part of more widespread . Br Heart J -analysis, J Am Coll Cardiol 2011;58;2241. 1976;38;472. 10. Wu MH, Chen HC et al Prevelence of congenital heart diseases 23. Cabera A,Martinez P et al.Double Chambered right ventricle at live birt at Taiwan. J Pediatt r 2010,156-782. Eur Heart J 1995 16;682. 11. Riggs T Sharp SE et al. Spontaneous closure ofatrial septal 24. Noonan JA Ehmke DA et al. Associated non cardiac malfor- defect in premature vs full term neonates. Peditr Cardiol mation in childrens with congenital heart diseases J Pediatr 2000,21;129. 1963;63;468.

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