LAPORAN KASUS

COR TRIATRIATUM SINISTER AT 34 DAYS OLD BOY

Adi Purnami, Eka Gunawijaya Departments of Child Health, Udayana University Medical School / Sanglah Hospital, Denpasar

ABSTRACT

Cor triatriatum is a rare congenital cardiac anomaly. In this malformation, an anomalous septum divides the left or right atrium into two chambers. Cor triatriatum represent 0.1% of all congenital cardiac malformations and may be associated with other cardiac diseases in as many as 50% of cases. The natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chamber. If the communicating orifice is small, the patients is critically ill and may succumb at a young age (usually during infancy) due to congestive failure and pulmonary edema. If the connection is larger, patients may present in childhood or adolescent periode with a clinical pictures similar to that of mitral stenosis. We report a case of cor triatriatum in a 34 days old boy with chief complaint of shortness of breath and his lips became blue when crying since 2 week prior admission. The diagnosis was based on clinical features, chest radiography and echocardiography. The only treatment is surgical correction. Patient had total obstructive of pulmonary veins drain to left heart and partial AVSD so the prognosis is poor becaused he died one day after hospitalized. [MEDICINA 2014;45:65-70].

Keywords: cor triatriatum, children

COR TRIATRIATUM SINISTER AT 34 DAYS OLD BOY

Adi Purnami, Eka Gunawijaya

Bagian/SMF Ilmu Kesehatan Anak Fakultas Kedokteran Universitas Udayana/ Rumah Sakit Umum Pusat Sanglah, Denpasar

ABSTRAK

Cor triatriatum merupakan kelainan bawaan yang sangat langka. Pada kelainan ini jaringan ikat fibrus akan membagi atrium kanan atau kiri menjadi 2 bagian. Angka kejadian hanya sebesar 0,1% dari seluruh kelainan jantung bawaan dan biasanya ditemukan bersama-sama dengan kelainan jantung lainnya. Perjalanan klinis tergantung dari hubungan antara bilik atas dengan bilik bawah dari atrium yang terbagi. Bila lubang penghubungnya kecil, keluhan mulai muncul sejak lahir dan biasanya meninggal saat masa bayi. Bila penghubungnya besar, keluhan akan muncul pada usia anak atau dewasa dengan gejala klinis menyerupai stenosis mitral. Kami melaporkan kasus pada bayi usia 34 hari yang datang dengan keluhan sesak napas dan kebiruan pada bibir bila menangis. Diagnosis ditegakkan dari klinis, foto dada, dan ekokardiografi. Satu-satunya terapi adalah koreksi melalui pembedahan, tapi kasus meninggal sebelum pembedahan dilakukan. [MEDICINA 2014;45:65-70].

Kata kunci: cor triatriatum, anak

INTRODUCTION chamber that is related to the left defect depends on the size of the atrial appendage and the mitral communicating orifice between the or triatriatum is a rare valve orifice. However, variable upper and lower atrial chamber.7 C congenital cardiac types of subtotal cor triatriatum If the communicating orifice is anomaly in which a fibromuscular are also noted, with only right or small, the patients is critically ill membrane divides the left or right left pulmonary veins draining into and may succumb at a young age atrium atrium into two the upper chamber. Cor (usually during infancy) due to chambers.1,2 In its most common triatriatum represent 0.1% of all congestive heart failure and form, about 54% are cor congenital cardiac malformations pulmonary edema. If the triatriatum sinister,3 the left and may be associated with other connection is larger, patients may atrium is divided into an upper cardiac disease in as many as 50% present in childhood or adolescent chamber that receives the of cases.5,6 periode with a clinical pictures pulmonary veins and a lower The natural history of this similar to that of mitral stenosis.

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Approximately 75% of patients die 190x/minutes regular in rhytim. midclavicular line sinistra with in infancy (generally from His axillary temperature was 36.20 grade III/6. Abdominal pulmonary hypertension) if the C. Body weight was 3.7 kg examination revealed no distended defect is unrepaired.7 If the compares to ideal body weight abdomen with normal bowel communicating between the patient’s nutritional status was sound. Liver was palpable 1/2-1/2, proximal and distal chambers is considered as mild malnutrition. with blunt edges and soft, spleen not restrictive or if an atrial septal The oxygen saturation was 64% in was not palpable. Capillary refill defect allows decompression of the room air and increase to 90% with time was more than 3 minutes with hypertensive left atrium, the oxygen of 10 lpm via oxygen hood. cutis marmorata and cyanosis seen prognosis is significantly There were no pale on his extremities. improved.7,9,10 conjungtiva nor icteric sclera were The result of the laboratory The only treatment is surgical noted. His lips looked cyanosis. investigation showed WBC 12.2 K/ correction. Most postoperative There was no abnormalities on uL, Hb 13.8 g/dl, HCT 42%, PLT deaths occur within the first 30 ear. Nasal flare was noted in this 162 K/uL, CRP 0.428 mg/dl, blood days. The early mortality rate in patient. There was no enlargement sugar 43 mg/dl, sodium 135 mmol/ each large series was consistently of lymph nodes or nuchal rigidity. L, potassium 4.619 mmol/L, 15-20%. Early deaths had a higher On chest examination, we found chloride 95.63 mmol/L, calcium rate of associated severe cardiac the chest wall shape was normal 5.905 mmol/L. Arterial blood gas anomaly.3 Long term results are and symetric. There was showed metabolic acidosis and excellent, with long term survival subcostals, intercostals and respiratory acidosis with pH 7.04, of 80-90% in patients surviving suprasternal chest indrawing. The pCO2 35 mmHg, pO2 67 mmHg, surgery.6 breath sounds was HCO3 9.50 mmol/L, TCO2 10.6 The purpose of this case report broncovesicular, with rales in lung mmol/L, BE -20 mmol/L, SaO2 was to keep us more alert to this base without wheezing. There was 81%. disease, to know the clinical no precordial bulging. Ictus cordis Chest radiography revealed symptoms, diagnosis, treatment, was palpable but not accentuated cardiomegaly with cardiothoracic and prognosis. on the 3rd intercostals space on left ratio (CTR) of 72%, enlargement mid-clavicular line. Thrill was not of right atrium, right ventricle THE CASE palpable. There was right with prominent pulmonal segment JN, a 34 day old boy was ventricular (RV) heave. and increased of pulmonary referred from another public Auscultation revealed vascular marking (Figure 1). hospital to the Pediatric accentuation of the pulmonary Echocardiograpraphy revealed Emergency Department at second sound with gallop, systolic cor triatriatum (Figure 2) 2 Sanglah Hospital on November murmur on the 3th parasternal line chambers of left atrium (LA), with 23th, 2010 due to shortness of sinistra and diastolic murmur on functionally intact intra LA septal, breath. Mother noted that the the 2nd-3rd intercostals space on without persisten foramen ovale patient had shortness of breath since 2 week prior to admission and worsen since1 week prior to admission. The other noted that he was often became blue in his lips while he was crying since 2 week prior admission. Feeding difficulties also noted same time with shortness of breath, and as a result he had weight lost in two weeks. There was no cough, fever, and common cold within two weeks was noted. He was born term with no cry after delivered. His birth weight was 3200 grams. No visible abnormalities were found. Upon admission (November 23th 2010) according to physical examination, he was looked pallor and iritable. Respiration rate was 78x/minute with the heart rate Figure 1. Chest radiography.

66 • JURNAL ILMIAH KEDOKTERAN Cor Tria Triatum Sinister At 34 Days Old Boy| Adi Purnami, dkk.

He was hospitalized in Pediatric Intensive Care Unit and treated with oxygen using continous positive airway pressure (CPAP), IVFD D51/4 NS 320 ml/ day with the rate 12 micro drips/ minute, intravenous furosemide at 4 mg every 12 hours, dopamine 5 micrograms/kg/minute. And we planned to refer to cardio-thoracic- vasculer surgeon for intra-atrial septectomi procedure. Unfortunately the patient died on November 24 2010, one day after hospitalisation.

DISCUSSION Cor triatriatrum sinister are more common form than cor triatriatum dextrum.3 In cor Figure 2. Echocardiography showed cor triatriatum, dilated RV, dilated triatriatum sinister the left atrium RA, smallish LV. is divided into an upper chamber that receives the pulmonary veins and a lower chamber that is related to the left atrial appendage and the orifice.1,2 Cor triatriatum represents 0.1% of all congenital cardiac malformation,2,3 and may be associated with other cardiac defect in as many as 50% of cases. Examples of associated cardiac defects include , persistent left superior vena cava with an unroofed coronary sinus, partial anomalous pulmonary venous connection, ventricular septal defect, and more complex cardiac lesions, such as , atrioventricular canal, and double outlet right ventricle.3 Depending on the presessence and localization of an atrial septal defect, there are Figure 3. Echocardiography showed, small aorta and large RV. four types of cor triatriatum (Figure 4). Type 1 (intact inter- atrial septum), type 2 (ASD to (PFO)/atrium septal defect II), systolic function was normal with upper atrial chamber), type 3 dilated right atrium (RA) & right ejection fraction (EF) 77.2%. (ASD to lower atrial chamber), and ventricle (RV), smallish left Based on clinical type 4 (ASD to both chamber). In ventricle (LV), small Ao (Figure manifestation, radiologic, and our case, the diagnosis was cor 3), no persistent ductus arteriosus echocardiography investigation, triatriatum sinister with total (PDA) severe pulmonary the final diagnosis was congestive obstruction of pulmonary veins hypertension (PHT) (pulmonal heart failure functionally drain to left heart, and its present arteri pressure estimation 99 Ross IV + cor triatriatum with during infancy (1 month). We also mmHg), tricuspid regurgitation total obstructive of found partial AVSD and large ASD and partial atrioventricular septal pulmonary veins drain to left I in our case, which the anatomical defect (AVSD). Left ventricular heart + partial AVSD was type 3 with ASD to lower atrial chamber.

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at Sanglah Hospital due to shortness of breath. Mother noted that the patient’s have shortness of breath since 2 week prior to admission and worsening since I week prior to admission. The other noted that he was often became blue in his lips only while he was crying since 2 week prior admission. He had feeding difficulties since the shortness of breath occur, and as a result he had weight lost in two weeks. There was no cough, fever and common cold within two weeks were noted. Despite the intracardiac defect, no characteristic murmur or pathognomonic physical Figure 4. Anatomical types of cor tri atriatum. characteristics are present. Signs of pulmonary venous obstruction The natural history of this hypertensive left atrium, the and pulmonary hypertension are defect depends on the size of the prognosis is significantly present. A right ventricular lift and communicating orifice between the improved. In our case, the accentuation of pulmonary second upper and lower atrial chambers. patient can not survive from cor sound are frequent and may be If the communicating orifice is triatriatum with total obstructive accompanied by an early diastolic small, the patient is critically ill pulmonary veins drain to left heart murmur of pulmonary and may succumb at young age and died at 35 day old, just one day insufficiency. Rales may be (usually during infancy) due to after hospitalization. present in the lung base. A soft congestive heart failure and Cor triatriatum is essentially continous murmur may be present pulmonary edema. If the conection a form of left atrial inflow due to flow across the membrane. is larger, the symptoms will obstruction and presents with A murmur at the left sternal present during childhood or signs and symptoms of pulmonary border is heard in patients with an adolescents with a clinical picture venous obstruction. Most patients atrial septal defect and a left-to- similar to that of mitral stenosis. presents during infancy with a right shunt. A diastolic rumble of Cor triatriatum may also be an restrictive opening in the mitral stenosis at the apex is incidental finding when it is non membrane. These infants is generally not heard with cor obstructive. In our case, the usually present with evidence of triatriatum. In our case, there symptom appeared at 1 month of low cardiac output, including was subcostal, intercostals, and age, with congestive heart failure pallor, diminished peripheral suprasternal chest indrawing. The and pulmonary edema because pulses, and tachypnea and breath sounds were there was total obstruction of tachycardia. Feeding difficulties, bronchovesicular, with rales in pulmonary vein drain to left heart, poor weight gain, and respiratory lung base without wheezing. Ictus it is means there is functionally distress are common. Presentation cordis was palpable but not no communicating orifice between later in life is less classic; however, accentuated with impulse on the upper and lower atrial chamber. when the patient becomes 3rd intercostals space in left mid Meaning, may be there was symptomatic, evidence of clavicular line. Thrill was not restrictive communicating but the pulmonary venous obstruction palpable but there was RV heave. flow can not be seen on color dopler predominates. In these patients, Auscultation revealed there was echocardiography. the membrane may become accentuation of the pulmonary Approximately 75% of patients calcified with its orifice becoming second sounds with gallop, systolic die during infancy (generally from smaller or the patient may develop murmur on the 3th parasternal pulmonary hypertension) if the mitral valve insufficiency. These line sinistra and diastolic murmur defect is unrepaired. If the patients may also present with on the 2nd-3rd intercostals space on communication between the arrhythmias secondary to an left parasternal line grade III/6. proximal and distal chambers is enlarged, hypertensive atrium. In Other clinical manifestation is not restricrive or if an atrial septal our case, patient referred from caused by low cardiac output such defect allows decompression of the another public hospital to the as: pallor, tachypnea, tachycardia, Pediatric Emergency Department 68 • JURNAL ILMIAH KEDOKTERAN Cor Tria Triatum Sinister At 34 Days Old Boy| Adi Purnami, dkk. and diminished peripheral pulses. Echocardiography is often prognosis. If the connection Children with cor triatriatum are sufficient for diagnosis and is the between the proximal and distal typically small, suffering from poor diagnostic modality of choice. chamber is not restrictive or if an weight gain. Patient may be Demonstration of the membrane atrial septal defect is present, dyspneic with a history of frequent by two-dimensional imaging, prognosis is improved. In our pulmonary infections. They may evaluation of turbulen flow on case, the patient had total have sign of right-sided heart colour Doppler and determination obstructive of pulmonary veins failure, including distended of any gradient across the drain to left heart and partial peripheral veins, increased membrane on continous wave AVSD so the prognosis is poor jugular venous pressure (JVP), Doppler can be performed with becaused he died one day after and hepatomegaly. Signs and echocardiography. Coexisting hospitalized. symptoms of pulmonary congenital anomalies can also be hypertension may be severe. In well characteristic by SUMMARY our case, patient revealed echocardiography. In our case, We report a case of Cor tachypneu with the the respiration we found the membrane dividing Triatriatum at 34 days old boy. rate was 78x/minutes, low the left atrium as the The definitive diagnosis was peripheral oxygen saturation, it characteristic of cor triatriatum established by clinical feature, was 64% in room air and increase sinistra. It can also showed large chest radiography and to 90% with oxygen 10 lpm via atrial septal defect, partial AVSD echocardiograpy. Surgical is the oxygen hood. Patient also had poor as a coexsisting cardiac congenital only treatment, but unfortunately weight gain, because the body anomalies in cor triatriatum. the case died one day after weight was 3.7 kg, compares to Surgery is the treatment of hospitalization. ideal body weight 3.9 kg, patient’s choice. Open correction is nutritional status was considered currently preferred over closed REFERENCES as mild malnutrition. Liver was (percutaneous) procedure. The 1. Park MK. 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