Divisi Kardiologi Pediatrik & Penyakit Jantung Bawaan Departemen Kardiologi & Kedokteran Vaskular Fakultas Kedokteran Universitas

Indonesia

 Inisial Jenis Kelamin Usia Alamat Pembiayaan MRS

: An. AY : Laki-laki : 10 tahun 1 bulan : Jakarta : Gakin DKI : 9 Februari 2010

Perut semakin membesar

Riwayat Perjalanan Penyakit

April 2009 Juni-Jan 2009
Keluhan perut membesar, sesak (-) Dirawat 4x di RSPP dan dilakukan penghisapan cairan perut 2x. Terakhir dirawat bulan Nov 2009: dikonsulkan ke SpJP dikatakan krn kelainan jantung. Pasien pulang dalam keadaan perut masih agak besar. Pasien menjalani rawat jalan. Terapi: Lasix 1x40mg, Letonal 1x25mg, digoxin 1x1tab.

Feb 2010
R U J U K P J N H K

Riwayat batuk lama disertai dahak, nafsu makan kurang. Sesak (-) Dirawat dua minggu keluhan demam disertai batuk dan sesak. Pemeriksaan CXR dan dahak TB paru mendapat OAT Dua bulan kemudian, perut mulai membesar. OAT dihentikan.

Keluhan perut semakin membesar, lemas, tidak nafsu makan dan mata tampak kekuningan Dirujuk ke PJNHK dg D/ Perikarditis konstriktif

R A W A T

Riw kehamilan: ANC teratur di dokter, ibu sakit berat (-), perdarahan (-), keputihan (-), minum jamu/obat(-) Riw persalinan: spontan, aterm, BL 3300 gram, langsung menangis (+), biru (-) Pasien merupakan anak ke-3 dari 4 bersaudara, kakak dan adik pasien sehat. Usia ibu saat hamil 43 tahun dan ayah 42 tahun. Ayah pasien sering batuk tanpa demam dan disertai dahak. Ayah pernah mendapat OAT 8 bulan yll setelah diperiksa rontgen dan dahak, namun obat dihentikan sendiri.

KU: CM, tampak lemah TD 86/48, HR 115 x/mnt, RR 24 x/mnt, Sat 100% BB 27 kg, lingkar perut: 74 cm Mata: konjungtiva -/-, sklera ikterik +/+ Leher: JVP 5+2 cmH2O, Kusmaull sign (+) Thoraks: simetris statis dinamis Jantung: Iktus kordis di ICS IV 1 cm medial midklavikula, BJ I N, BJ II N, murmur (-), gallop (-), friction rub (-), pericardial knock (-) Paru: vesikuler, rhonki -/-, wheezing -/Abd: buncit, tegang, shifting dullness (+), heparlien sulit dinilai Ekstremitas: akral hangat, edema -/-

Hb Ht Leukosit Trombosit LED CRP GDS Ureum Creatinin Na K Ca total Cl

: 13,7 : 41 : 4080 : 217000 :6 :4 : 77 : 31 : 0.7 : 135 : 2,8 : 2,2 : 94

Protein Albumin Globulin Bilirubin total Bilirubin direk SGOT SGPT

: 5,0 : 2,4 : 2,6 : 0,74 : 0,34 : 52 : 11

Bilirubin indirek : 0,39

ST, QRS axis N, QRS rate115 x/mnt, PR interval 0,12, QRS durasi 0,08, low voltage pada limb & precordial leads, ST changes (-), T bifasik-inverted pada lead II, III, aVF, V2-V6.

Inspirasi kurang, asimetris, eksposure cukup CTR 48 %, segmen aorta normal, segmen pulmonal normal, pinggang jantung(-), apex downward, kongesti (+), infiltrat (-), efusi pleura (-)

Situs solitus, IVC >>>, RA besar, All PV to LA Fungsi LVEF 54% (Simpson) Diskinesia IVS jerking movement posterior, mid anterior, anterolateral menempel pada perikardium tak bergerak IVS intak TR mild TVG 10 mmHg Doppler : E/A >1, Mitral inflow E-A variation > 25% saat inspirasi & ekspirasi

Kontraktilitas RV , TAPSE 8 mm Kesimpulan: pericarditis konstriktif, fungsi RV terganggu.

 Dx

fungsional Dx anatomis Dx etiologi

Masalah:

: right heart failure : perikarditis konstriktif : susp TBC perikardium

Asites Hipoalbumin Hipokalemia Gangguan fungsi hati

 R/jangka

panjang R/jangka pendek

:perikardiektomi :perbaikan KU, eksplore ke arah TBC, evaluasi ggn fungsi hati lebih lanjut

Tatalaksana:

Rawat IW Anak pemasangan vena dalam Koreksi albumin Koreksi kalium

Follow Up
10 Feb 2010
Balans cairan +474 ml USG abdomen:  sirosis hepatis (kardiak) dgn asites massif,  CKD kanan DD/: contracted ginjal kanan,  cholesistitis ec asites. demam, batuk berdahak, muntah Paru: rhonki basah kasar lobus tengah - bawah paru kanan Leu 6230, LED 6, CRP 7 Tatalaksana: Aldactone 1 x 50 mg mg Lasix drip 5 mg/jam Diet enteral per NGT hepatosol 8 x 100 mL (150 kkal) Diet per oral sesuai keinginan pasien (rendah garam III) Total cairan 1100 mL (80%) R/ perikardiektomi 18 Feb 2010 BC -844 aldactone 1 x 50mg Lasix 3mg/jam Ranitidin 2x1/2amp Vometa 3x1tab Cefotaxime 2x500mg Cek kultur darah Tes Mantoux (-),BTA 3x(-), PCR TB darah(-),HBsAg (-) Urin rutin: kuning jernih, epitel (+), pH 6, BJ 1010, eri (-), leu (-), silinder (), kristal (-), bakteri (-), bilirubin (-), keton(-), glukosa (-), protein (-), urobilinogen <1, nitrit (-).

12 Feb 2010

13 Feb 2010

Follow Up
16 Feb 2010

18 Feb 2010

22-28 Feb 2010

Hb 12,9 / Leu 10100 / LED 6 /CRP 27/ Ht 38 / alb 4,0 / Na 131 / K 2,8 Kultur darah (-)

Demam menghilang R/ perikardiektomi Pasien demam tunda operasi ECG: stqa Leu 18800

9810

R/ perikardiektomi 1 Mar 2010

Berat badan
28 27 26 25 24 Berat badan 23 22 21

Lingkar perut
76 74 72 70 68 66 64 62 60 Lingkar perut

Temuan intraoperatif :

Pericardium menebal, tegang, kekuningan. RA dilatasi. Perkijuan dan perlengketan di seluruh permukaan jantung eksisi dan release restriksi perikard. Dibebaskan perlengkatan yang hebat di aspek lateral, anterior, serta inferior. Sempat terjadi bleeding ketika membebaskan permukaan lateral kanan jantung, bleeding dari RA kanulasi dari arteri dan vena femoral, mesin jantung dijalankan perdarahan dari RA diatasi.

Dilakukan pengambilan bahan untuk kultur dan pemeriksaan PA

Echo post op (1 Mar 2010):

Pericardial effusion (-), IAS bulging ke LA, LVEF 60% dengan jerking movement septal, TVG 15 mmHg, RV fungsi TAPSE 1,2 cm, E/A >2, restrictive filling.

 Konsul

pulmonologi:

INH 1 x 250mg Rifampicin 1 x 350 mg Pirazinamid 2 x 250 mg Etambuthol 1 x 400 mg Prednison 3 x 4mg (1 bulan tappering off) Ventolin expectorant 3 x cth 1

An 10 tahun dengan right heart failure dengan sirosis hepatis ec perikarditis konstriktif susp TB, telah dilakukan tindakan perikardiektomi yang didapatkan hasil perikardium menebal dengan perlengketan pus pada kavum perikardium, diambil bahan untuk pemeriksaan kultur dan histologi PA. Pada pasien juga diberikan terapi koreksi albumin dan kalium, aldactone 1x50mg, lasix drip 3 mg/jam, dan terapi OAT.

Fibrous sac surrounding heartdense network of collagen fibres Serous membrane two continuous layers separated by a small amount of fluid lubricant (10-20mls ) Layers are : Visceral is inner layer (epicardium) Parietal is continuous with diaphragm and outer walls of great arteries

 Constrictive

pericarditis is a condition in which a thickened, scarred, and often calcified pericardium limits diastolic filling of the ventricles. acute pericarditis, cardiac trauma and surgery, radiation therapy, renal failure, and connective tissue diseases are most common causes

Idiopathic,

Guidelines on the Diagnosis and Management of Pericardial Disease. European Heart Journal 2004

Guidelines on the Diagnosis and Management of Pericardial Disease. European Heart Journal 2004

Guidelines on the Diagnosis and Management of Pericardial Disease. European Heart Journal 2004

Solomon SD. Essential Echocardiography

Guidelines on the Diagnosis and Management of Pericardial Disease. European Heart Journal 2004

 Supportive

care Symptomatic patients require admission and pericardiectomy

Pericardiectomy for Pericarditis in the Pediatric Population

Ann Thorac Surg 2009;88:1546-1550

Conclusions: In properly selected pediatric patients, complete pericardiectomy can be performed with good outcomes. Although the etiology of pericardial irritation is frequently elusive, resolution of symptoms can be expected in most patients. Confronted with medically refractory pericarditis, earlier consideration for pericardiectomy may be warranted.

A syndrome :

typical chest pain a pathognomonic pericardial friction rub specific ECG changes

Hurst's The Heart, 12th Edition

 Idiopathic Infection

Viral (Coxsackievirus type B, Echovirus) Tuberculosis Pyogenic bacteria

Non

infectious

post myocardial infarction Neoplastic Radiation induce Connective tissue diseases Drugs

Lilly L.S, 2007, Pathophysiology of Heart Disease

PERICARDITIS

Diffuse ST segment elevation with concavity upward in most leads Diffuse P-R interval depression in most leads T waves are upright (in contrast to ischemia)

 Pericardial

friction rub :

superficial the sound of walking on dry snow / the squeak of a leather saddle between the lower left sternal edge and the cardiac apex

 Idiopathic

or viral pericarditis limiting disease (1 3 weeks) require bed rest initial attack

self

Patients

Colchicine NSAID

(aspirin 650 mg/3 hours, ibuprofen 300 to 800 mg/6 hours) relieving symptoms of chest pain 60 to 80 mg/d (caution) or antituberculosis

Prednisone Antibiotics

 Pericarditis Diagnosis But

is usually a benign disorder

relates to underlying cause

any cause can lead to an effusion and tamponade which can lead to death can also progress to pericardial constriction and heart failure

Pericarditis

 Accumulation

of transudate, exudate, or blood in the pericardial sac complication of pericardial disease

Common

Should

be sought in all patients with acute pericarditis

Very common after cardiac surgery Acute pericarditis Increased capillary permeability (severe hypothyroidism) Increased capillary hydrostatic pressure (CHF) Decreased plasma oncotic pressure (cirrhosis, nefrotic syndrome) Lymphatic obstruction (neoplasms)

 Asymptomatic

or symptomatic factors :

Volume of fluid Rate of fluid accumulates Complience of the pericardium

A

sudden increase of pericardial volume marked elevation of pericardial pressure slowly increase of pericardial volume without marked elevation of pericardial pressure

Asymptomatic

cardiac tamponade symptom

Compression symptom : Dysphagia (esophageal compression) Dyspneu (lung compression) Hoarseness (N. recurrent laryngeal compression) Hiccups (N. phrenicus stimulation) Soft heart sound Reduce intensity of friction rub Ewart sign dullness over posterior left lung

ECG

Low voltage Electrical alternans

CXR

May be normal silhouette Volume >250 ml enlarged cardiac silhouette (flask shape)

 Confirms Can

the clinical diagnosis

identify a small pericardial effusion (20 ml) the volume ventricular filling

Quantify

Determine Help

pericardocentesis

 If

the cause is known disorder Tx

underlying

Pericardiocentesis

Relieve symptom Establish etiology

A hemodynamic condition Equal elevation of atrial and pericardial pressures Pulsus paradoxus Arterial hypotension The fluid accumulation filling severely impairs heart

It is a medical emergency and must be treated promptly. Risk of death depends upon speed of diagnosis, treatment and underlying cause of the tamponade

Pericardial fluid pressure

Impaired diastolic filling

Venous pressure

Impaired SV

CO Systemic congestion Pulmonary congestion Hypotension

 IV

Fluid Bolus-improves RV filling and improves hemodynamics and diagnostic

Pericardiocentesis-therapeutic

Admission

to ICU or monitored setting