| ABSTRACT
Background and Purpose
Tetralogy of Fallot (ToF) and Secundum ASD in congenital heart disease are not strangers for practitioner, but if they co-exist with Anomalous Origin of Right Pulmonary Arteries (AORPA), it become a very rare case.This is the case report of Anomalous Origin of Right Pulmonary Arteries with Tetralogy of Fallot and Secundum ASD. Here, we will also describe how this congenital abnormalities that coexist together will effect the haemodynamic.
Case Description
A boy two months old, came to emergency department with chief complaint of dyspnea. He was referred from Pangkal Pinang Hospital with Bronchopneumonia and Suspect PDA. He had history of cyanosis since his birth, dyspnea, and cough for two weeks before visiting National Cardiovascular Centre Harapan Kita (NCCHK). In NCCHK hospital he was planned to run surgeries to fix these congenital abnormalities.
Discussion
The patient has been suffered from heart failure and pulmonary hypertension due to AORPA that made right lung had systemic blood pressure levels with the left pulmonary blood flow will be equal to systemic venous return. Even with asssociated Tetralogy of Fallot, the surgical risk is greater, however, successful surgical repair has been reported in those patients.
Conclusion
In this case report, the patient has this rare but serious condition is amenable to surgical repair particularly if operated on early in life. Direct anastomosis is surgical procedure of choice, because reconnection of the anomalous branch is important to avoid irreversible pulmonary vasculature changes and should not be deferred for corrective procedures of associated cardiac anomalies.
INTRODUCTION
AORPA is a rare congenital cardiac malformation, that should be distinguished from other heart defects associated with an anomalous blood supply to the lungs such as patent ductus arteriosus, major collaterals between the systemic and pulmonary circulation and truncus arteriosus.1
This type of cardiac malformation was described first by Fraentzel in 1868.1 Since then, almost 200 cases have been reported in the literature with a high mortality among patients not surgically treated.1 The largest reported series, held by Freedom RM et all, includes only 16 patients with this anomaly undergoing surgery over a 36-year period.2 Meanwhile in NCCHK, based on data of Medical Record, only 1 patient who was diagnosed either only AORPA or AORPA with coexisting ToF and Secundum ASD, from 2005 until now.3
In AORPA, pressure in the pulmonary artery arising from the aorta is systemic in almost all patients. As well, pressure in the pulmonary artery from the pulmonary trunk elevates until reaching a systemic or suprasystemic level. Because it has been shown that progressive pathologic changes occur in lung subjected to systemic pressures, surgical treatment is recommended as soon as a diagnosis is confirmed during the neonatal period.4
THE AIM OF CASE PRESENTATION
The aim of this case presentation is to present a rare case of AORPA with TOF and Secundum ASD.
CASE ILLUSTRATION
A boy, Ad, 2 months old, came to NCCHK with chief complaint of dyspnea. He was referred from Pangkal Pinang Hospital with Bronchopneumonia and Suspect PDA. He suffered from dyspnea in the last 2 weeks, accompany with cough. He had history of bluish that remedy with supplementation of nasal oxygen. There weren’t any feeding difficulties or failure to thrive in the past 2 months. His mother denied any history of seizure and any deterioration of cyanotic episodes.
Past ante natal care history, revealed that his mother in a good condition along pregnancy, doing routine check up every month to her midwife, she denied taking any herbal or unnecessary medicine. Delivery history, revealed that he was born aterm, assisted by midwife, bluish when borned, with birth weight 2600 grams. His mother’s age at pregnancy was 33 years old, his father’s was 38 years old.
On physical examination, he looked severely ill. Blood pressure was 124/99mmHg, heart rate was 168x/minute, temperature was 36,3°C, respiratory rate was 90x/minute, O2 Saturation was 60%, body weight 3100 grams. The conjunctiva was pale, sclera was not icteric, JVP was not elevated. First heart sound was normal, second heart sound was single and not splitted, both were heard with normal intensity, ejection systolic murmur grade 3/6 at second intercostal space on left sternal border, no gallop. Breath sound were vesiculair without rales, wheezing (+) was heard over both lung. There was undistended abdomen. Extremities were pale, warm and no edema.
Laboratory findings were Hb 8,6 gr/dl, Leucocyt 6620/ul, ESR 5mm/h, Hematocrit 27%, CRP 5 ml/L, Diff. count 0/1/0/68/19/12. Ureum 33 mg/dl, BUN 15,42 mg/dl, Creatinin 0,4 mg/dl, Random Blood Glucose 176 mg/dl, Blood gas analysis was: pH 7,22, PCO2 81mmHg, pO2 55mmHg, HCO3 32,I mmol/l, tCO2 32,4 mmol/l, aBE 4,4 mmol/l, SBE 4,9 mmol/l, SBC 28,3 mmol/l, O2 Saturation 88,4%. Sodium 135mmol/l, potassium 4,8 mmol/l, Total Calcium 2,0 mmol/l, Magnesium 1,9 mmol/l.
Chest X-Ray showed that CTR 70%, with left sided aortic knob, normal pulmonic segment, normal cardiac waist, upward apex, oligemic on left hemithorax, plethora on right hemithorax, no infiltrate, and cranialisation of vascular markings
ECG shown Sinus Rhythm, QRS rate 162x/m, axis QRS +120° (RAD), Normal P wave 0,04”, PR interval 0,08”, QRS duration 0,06”, R V3 + R V4 > 60mm. from ECG findings we concluded that the patients was in sinus rhythm with Biventricular Hypertrophy.
Echocardiography findings were Situs Solitus, AV concordance, Persistent Left Superior Vena Cava to dilated Coronary Sinus, all PV to LA, Secundum ASD 5mm L-R Shunt, Large VSD Perimembranous R-L Shunt, Overriding Aorta 50%, Moderate Pulmonal Stenosis Infundibulair grad 35mmHg, RPA from posterior sinistra of Ascending Aorta, no stenosis, with diameter of RPA 5mm, LPA (4mm) from dilated MPA (8mm) with original stenosis 2mm. Aortic, Mitral, Tricuspid valve well functioning, Left sided Aortic Arch, No Coarctatio Aorta, PDA (-). ECHO Summary : AORPA with ToF, bilateral SVCs.
The patient was diagnosed as AORPA, ToF and Secundum ASD. The medical therapy were Furosemide 2 x 3mg iv, Aldactone 1 x 6,25mg p.o, Aminophyllin 3 x 12 mg iv, Dexamethasone 0,5 mg iv t.i.d, Ranitidine 3 mg iv b.i.d, Ambroxol ½ cth p.o t.i.d, inhalation with combivent: NaCl 0,9% (1:2) q.i.d. On day 4th hospitalization, patient was intubated due to respiratory distress, until now patient still in Pediatric Intensive Care Unit, planned to undergo surgical intervention to fix his congenital cardiac abnormalities.
LITERATURE REVIEW
An anomalous origin of the right pulmonary artery from the ascending aorta (AORPA), a condition in which the right pulmonary artery arises from the ascending aorta in the presence of separate aortic and pulmonary valves, is an unusual congenital cardiac anomaly.4
Little is known about the embryogenesis and pathogenesis of this malformation, although an association has been suggested with the CATCH 22 syndrome complex, including Di George syndrome.1 Different authors have reported the important role of the neural crest cells in the development of the third and fourth pharyngeal pouch derivatives as well as the aortic arches and trunco-conal part of the heart.1 It is plausible that deletions of the chromosomic band 22q11 may cause some degree of neural crest derangement. Similar defects have recently been described in association with the fetal valproate syndrome, condition linked to Waardenburg syndrome and disorders of the neural crest cells migration. Aru et al. included this anomaly as an aortic arch anomaly hypothesizing that a failure of media fusion of the AOPA with the MPAT results in persistence of the aortic sac from which the AOPA originates. In the absence of the left sixth arch, the AOPA may not find connection to the MPAT and consequently, the aortic sac connection persists.1
The origin site of the AOPA is different. Some authors advocate the existence of two forms: a proximal one, the AOPA originates from the ascending aorta close to the valvar plane; and a distal one, the AOPA arises via a patent ductus arteriosus. The third form of presentation is the origin site close to the innominate artery or in extreme cases, the right AOPA may originate from the innominate artery itself. In six of the eight cases presented by Freedom RM et al, the AOPA originated from the respective proximal postero-lateral part of the ascending aorta. Usually, the origin is from the respective postero-lateral aspect of the ascending aorta.1
This anatomical anomaly causes the pressure in the abnormal pulmonary artery that arises from the aorta to be systemic in almost all patients. The pressure in pulmonary artery arising from right ventricle can increase to systemic or suprasystemic level.4 This phenomenon has not been fully explained, but several mechanisms have been postulated as the cause of bilateral pulmonary hypertension. These include high pulmonary blood flow , circulating vasoconstrictor substances, neurogenic crossover from an unprotected lung, and development secondarily after left ventricular failure. That’s some reasons that make patients characteristically present with early infantile respiratory distress and heart failure.
Tab 1. Cardiac abnormalities associated with AORPA.12
The AOPA may be isolated or associated with other congenital heart defects. Most of the reported cases presented some major associated cardiovascular defect such as tetralogy of Fallot, VSD, and patent ductus arteriosus. The right aortic arch has been reported in almost 50–75% of patients with left AOPA. Different cases have been reported with isolated malformation, although its incidence is very limited.1
Tetralogy of Fallot (TOF) is a frequently encountered congenital heart defect, and the detailed diagnostic criteria are well established. However, some discussion still occurs regarding treatment, particularly when surgical correction is contemplated for an infant. Other lesions might be associated with TOF; among them, the anomalous origin of the left pulmonary artery (LPA) from the ascending aorta is a rare one. It is well know that when occurring as an isolated lesion, the right pulmonary artery branch is usually involved and when associated with TOF, the left branch is more commonly affected.10 A study held by Edward W.K. Peng et al, said that AORPA with TOF or another group of patients with complex lesions associated with RVOTO were well at first presentation, but poorer functional outcome after surgery due to associated co-morbidities.8 Another study, held by Endo M, et al, at Tohoku University Hospital, between 1971 and 1988, with 7 patients of TOF with anomalous origin of unilateral pulmonary artery (AOPA) underwent intracardiac repair, concluded that TOF with AOPA should be corrected before the advance of PVOD in case PA originate from the ascending aorta, in order to prevent postoperative RV overload.
Because it has been shown that progressive pathologic changes occur in lung subjected to systemic pressures, surgical treatment is recommended as soon as a diagnosis is confirmed during the neonatal period.4 Early surgical intervention, preferentially before 12 months of age, is very important to prevent the development of irreversible pulmonary vascular disease. In patients in whom the anomalous origin is an isolated finding, surgical correction should be considered as soon as the diagnosis is established, preferentially within the first 6 months of life, due to the possibility of the early development of pulmonary vascular disease. Infants should be considered "operable" even when the resistance calculations predict otherwise as there appears to be a large component of immediately reversible pulmonary artery hypertension. With technical skills learned from arterial switch operations (for transposition of the great arteries), surgical repositioning of the RPA should be possible in nearly all cases without the use of a tube graft.7 The surgical correction may be performed with or without extracorporeal circulation, depending on the technical difficulties found during the procedure.5 Hypoplasia of the pulmonary artery connected to the right ventricle can protect the lung from pulmonary vascular disease. The results of the surgical correction in the past years have been promising, except in patients with associated cardiac malformations. Reconnection of this anomalous pulmonary artery should not be deferred to allow concomittant corrective procedures of other associated cardiac anomalies.5 In Marco Aurélio Santos’s et al case series, no death was observed during surgery or later. The palliative treatment of AORPA, such as pulmonary artery banding, and ligation of the associated ductus arteriosus and aortopulmonary shunt, is insufficient and only increases mortality substantially.5
DISCUSSION
In this patient that suffered from dyspnea accompany with cough, we can exclude possibilities of bronchopneumonia and PDA, from history taking, physical examination and others diagnostic modalities. His complains of dyspnea and cough came from existing heart failure. As we knew before that there were some mechanisms have been postulated as the cause of bilateral pulmonary hypertension in this patient such as high pulmonary blood flow and development secondarily after left ventricular failure. We can conclude that from the Chest X-Ray, we got plethoric view on right hemithorax with both lungs in cranialisation of vascular markings.
Considering, initially, the right lung, the right pulmonary artery will have systemic blood pressure levels and a flow that will depend on the resistance of the right pulmonary vascular system with elevated pressure.5
In isolated type of AOPA, left pulmonary lung flow will be equal to that of the systemic venous return. Variations in left pulmonary vascular resistance will be transmitted to the right ventricle, and, consequently, to the total cardiac output. The reasons for the elevation in left pulmonary vascular resistance are not yet well understood. How ever it seems not to be the result from an isolated increase in pulmonary flow, because patients with congenital absence of one lung also have one right ventricle managing the entire cardiac output, no major elevation in pulmonary vascular resistance is observed in their single lung.5 Except for the flow, the following factors can modify pulmonary vascular resistance: 1) pulmonary arterial anatomy, such as diameter, number and distribution of the vessels; 2) vascular tonus; 3) blood rheology, 4) Left Ventricle Failure with reactive Pulmonary Hypertension, and 5) concomitant ASD/VSD. This patients had no evidence of abnormal blood elements that could affect the characteristics of normal flow. The anatomical obstructions were not ruled out yet, by measuring pulmonary capillary wedge pressure. Despite all these considerations, the pulmonary blood flow seems to be the critical factor in the development of pulmonary vascular disease, because patients with tetralogy of Fallot as an associated defect show only unilateral alterations. Pulmonary hypertension has been described as starting early in most of the cases reported, and intervention had been mainly carried out in infancy, although there are rare cases of non-operated survivors in adulthood with apparently normal pressures in the lungs.6 This case also illustrates that echocardiography can diagnose these lesions. If the diagnosis is in doubt, angiography should be performed early.10,13
In patients with associated tetralogy of Fallot, the surgical risk is greater, however, successful surgical repair has been reported in those patients.5 Most commonly, the surgical correction has been achieved within the first month of age. Fontana et al. found a 30% 1-year survival in a series of 23 cases with AOPA not surgically treated. Successful correction of this anomaly in the first days of life, even in prematures, have been reported.1 Early repair is preferred to avoid pulmonary hypertension and irreversible pulmonary vascular disease. Serious pulmonary vascular disease has been observed in patients with AOPA as early as the third month of life.11 Various types of surgical techniques are employed for the repair of the anomalous pulmonary arteries, with the most frequently employed being direct anastomosis. Alternative techniques include end to end anastomosis with a synthetic graft, interposition with a homograft patch, aortic flap, etc.1 The results in the neonatal period have been promising, but due to the rarity of this lesion, it is difficult to predict the optimal timing or the best repair technique.
SUMMARY
A 2 months old boy with AORPA, ToF and Secundum ASD, referred from Pangkal Pinang Hospital, has been hospitalized due to respiratory distress. At present the patient is still in Intensive Care Unit due to his heart failure and pulmonary hypertension, and he was planned to undergo surgical intervention to fix his congenital cardiac abnormality as soon as possible as his condition is well enough for surgery.
REFERENCES
1. Edvin Prifti et al, Postoperative Outcome in Patients with Anomalous Origin of One Pulmonary Artery Branch from The Aorta, Elsevier Science NL, Eur J Cardiothorac Surg 2003;24:21-27
2. Abu-Sulaiman R.M., Hashmi A., McCrindle B.W., Williams W.G., Freedom R.M. Anomalous origin of one pulmonary artery from the ascending aorta: 36 years' experience from one centre. Cardiol Young 1998;8(4):449-454
3. Data from Medical Record of National Centre of Cardiovascular Harapan Kita, Jakarta, 2010
4. Noriyoshi Kajihara, MD et al., Surgical Results of Anomalous Origin of the Right Pulmonary Artery From the Ascending Aorta Including Reoperation for Infrequent Complications. Ann Thorac Surg 2008;85:1407-1411.
5. Marco Aurélio Santos et al., Anomalous Origin of One Pulmonary Artery from the Ascending Aorta. Surgical Repair Resolving Pulmonary Arterial Hypertension. Arquivos Brasileiros de Cardiologia - Volume 83, Nº 6, Desember 2004:503-506
6. Rasool AHG et al., Anomalous right pulmonary artery from the aorta. Images Paediatr Cardiol 2006;26:1-4
7. B Agarwala; J D Waldman; M Sand; W A Loe; D G Ruschhaupt, Aortic origin of the RPA: immediate resolution of severe pulmonary artery hypertension by surgical repair. Pediatric Cardiology Journal, 1994; vol.15:0172-0643
8. Edward W.K. Peng, Ganesh Shanmugam, Kenneth J.D. Macarthur, James C.S. Pollock. Ascending aortic origin of a branch pulmonary artery: surgical management and long-term outcome. Eur J Cardiothorac Surg 2004;26:762-766
9. K. Fujiwara, T. Takeuchi, H. Suzuki and S. Uemura. Tetralogy of Fallot with Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta and Hypoplastic Left Pulmonary Artery Pediatric Cardiology Volume 26, Number 5 / October, 20050172-0643
10. Fernando Amaral, Mônica A. C. Teixeira, João A. Granzotti, Paulo Henrique Manso, Walter V. A. Vicente, Anomalous Origin of the Left Pulmonary Artery from the Ascending Aorta. Successful Surgical Correction in an Infant with Fallot's Tetralogy, Arquivos Brasileiros de Cardiologia vol.79 no.5 São Paulo Nov. 2002
11. S Sugimoto; S Kikuchi; H Oosawa; Y Hachiro; N Takagi; T Ab. A reoperation for anomalous origin of right pulmonary artery: report of a case. Surgery today Volume: 31 ISSN: 0941-1291
12. Santos M, Azevedo VM. Anomalous Origin of One Pulmonary Artery from the Ascending Aorta. Surgical Repair Resolving Pulmonary Arterial Hypertension Arquivos Brasileiros de Cardiologia 2004; 83: 503-6
13. Reyes de la Cruz L, Vizcaìno Alarcón A, Arévalo Salas A, Espinosa Islas G, Bolio Cerdán A, Arteaga Martìnez Mrch, Echocardiographic diagnosis of anomalous origin of one pulmonary artery from the ascending aorta. Cardiol Mex. 2003 Apr-Jun;73(2):115-23.
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