Pembimbing: dr.Alifah A., M.Sc, Sp.

A
Penyaji: dr. Braghmandaru Adhi Bhaskara

IDENTITAS

Nama Tgl Lahir Alamat No.RM

: By. Ny. DF : 8 Januari 2014 : Krapyak : 74.03.43

MASALAH UTAMA

Takipneu, Ibu DM

Riwayat Penyakit Sekarang:

1 minggu yll,lahir bayi perempuan dari ibu P3A0, secara spontan, saat lahir bayi tidak menangis, tidak bergerak, sianosis seluruh tubuh, Apgar score 3/7, dilakukan resusitasi s/d oksigen aliran bebas. Air ketuban hijau keruh, meconium (-).
Ibu bayi adalah penderita hipertensi, tidak kontrol rutin, tensi dikatakan 130-140an, selama hamil tensi 160an. Diketahui DM saat mondok di RSS. ayah bayi meninggal 6 bulan yll, dikatakan sakit jantung(?)

RIWAYAT KELUARGA
Tidak ada riwayat atopik pada keluarga Tidak ada riwayat penyakit kongenital lain

pada keluarga Curiga ada penyakit jantung di keluarga Terdapat riwayat hipertensi pada ibu pasien Terdapat riwayat DM pada ibu

Silsilah keluarga

45th,peny.jantung (+),DM(-)

27th, obese(+), Hipertensi (+),DM(+)

RIWAYAT KEHAMILAN DAN KELAHIRAN

ANC:rutin kontrol di bidan puskesmas, rutin minum vitamin, hamil ke-3, riwayat trauma -, minum jamu -, minum obat-obatan -, sakit hingga mondok -, perdarahan -, hipertensi (+) NC:lahir di RSS, spontan, BBL: 5280 gram, umur kehamilan 38minggu, PB 54cm , lingkar kepala 38cm,lingkar dada 38.5cm, LLA 14cm, tidak menangis, biru +, kekuningan PNC:NICU Kesan: simpulan riwayat kehamilan,dan persalinan kurang baik

Anamnesis sistem

Demam (-)
Sistem CNS: kejang (-), penurunan kesadaran (-) Sistem respirasi: sesak (-), batuk (-), pilek (-) Sistem kardiovaskular: sesak (+), kebiruan (-),

bengkak (-) Sistem gastrointestinal: meconium(+), intake SF 12x10-15cc Sistem genitourinaria : miksi (+) Sistem integumentum:sianosis (-), ikterik (-) Sistem muskuloskeletal: deformitas (-),

Kesan Umum: gerak aktif, kesan gizi cukup

Tanda Utama:
HR 125x/menit, R 65x/menit, retraksi (-) T 36,8C, suhu aksila

SpO2: 97% (NCPAP, FiO2 4lpm)

Pemeriksaan Antropometri BB 5280 g,

TB 54 cm, LLA 14 cm, LK 38 cm, LP 36 cm, LD 38.5cm

LEHER JVP meningkat (-), limfonodi tidak teraba

PERUT DP//DD,BU (+) dbn,Hepar& Lien tak teraba ANOGENITAL

perempuan

ANGGOTA GERAK Gerakan bebas, akral hangat, perfusi baik CRT<2, edema (-), broad thumbs(-)

Pemeriksaan Jasmani
KULI T

petechiae (-), kering

OTOT

Atrofi (-), tonus menurun

TULANG

Deformitas (-), Fraktur (-)

SENDI

Deformitas (-), dislokasi (-)

Kepala Ukuran : normal Mata : konjungtiva anemis, sklera tak ikterik

Pulmo

Insp. Palp.

Cor

: simetris, KG (-), retraksi (-) : stem fremitus kanan = kiri Perk. : sonor Ausk. : vesikuler (+) N, menurun pada SIC VI kebawah RBK -/+,RBB -/-, wheezing -/: Insp. : IC tak tampak
Palp. : IC teraba di SIC VI LMCS, thrill (-), RV heaving (-) Perk. : kesan kardiomegali (+) Ausk. : S1N S2 split tak konstan, reguler, bising sdn

LABORATORIUM Hb AL AE AT Hmt : 14.1 : 25.9 : 4.29 : 277 : 44 Alb BUN Cre GDS Tbil Dbil Na K Cl Ca : 3.28 : 31.7 : 12.3 : 47 :7.33 : 0.55 :142 : 3.92 : 103 : 1.73

S L M E B

: 54 : 37 : 6.1 : 0.4 :0

CRP

: <10

 BBLB, CB, BMK, spontan, ibu PEB, DM Sepsis neonatorum Hipoglikemia neonatus Problem: Kardiomiopati?

Echo (8januari 2014)

Situs solitus, AV-VA concordance Muara vv pulmonales dan sistemik normal IVS dan LVPWD menebal ringan (IVS 6mm, LVPWD 5.7mm, LVIDd 16 mm) IAS dan IVS intak Katup-katup baik Kontraaktilitas LV baik Arc.Ao ada di kiri, tak tampak CoA maupun PDA Kesimpulan : LVH konsentrik ringan Saran: propanolol 0-5-1mg/kgBBhari

Infants of Diabetic Mothers

Prevalensi: 1.3% dari seluruhan kehamilan

Patofisiologi
DM mempengaruhi multi-organ Prevalensi malformasi kongenital padaa bayi dengan ibu DM 6-9% (3-4x > bayi biasa) Neural tube defect(anencephali/myelomeningocele), congenital heart defect, sacral dysgenesis/agenesis adalah yang tersering

Congenital heart defect, cardiomyopathy, persistent pulmonary hipertensi of the newborn Tersering: VSD, TGA, truncus arteriosus, tricuspid coarc aorta

CLINICAL MANIFESTATIONS

1. The history usually reveals gestational or insulin-dependent diabetes mellitus in the mother. The patient often has a history of progressive respiratory distress with tachypnea (80 to 100 breaths/minute) from birth. 2. These large-for-gestational-age babies are often plethoric and mildly cyanotic and may have tachypnea and tachycardia (>160 beats/minute). Signs of congestive heart failure (CHF) with gallop rhythm may be found in 5% to 10% of these babies. The patient may have a systolic murmur along the left sternal border, which may be caused by an outflow tract obstruction or an associated defect. 3. Chest x-ray films may reveal a varying degree of cardiomegaly. Pulmonary vascular markings are normal or mildly increased because of pulmonary venous congestion. 4. The ECG is usually nonspecific, but a long QT interval caused by a long ST segment secondary to hypocalcemia may be found. Occasionally, RVH, LVH, or biventricular hypertrophy (BVH) may be seen. 5. Echo may show the following: a. The ventricular septum is often disproportionately thicker than the LV free wall, but even free walls are thicker than normal (see Fig. 18-7 ). The degree of asymmetrical septal hypertrophy has no relationship to the severity of the maternal diabetes. b. Supernormal contractility of the LV and evidence of LVOT obstruction appear in about 50% of infants with cardiomyopathy. c. Rarely, the LV is dilated, and its contractility is decreased.

General supportive measures are provided, such as intravenous fluids, correction of hypoglycemia and hypocalcemia, and ventilatory assistance, if indicated. 2.In most cases, the hypertrophy spontaneously resolves within the first 6 to 12 months of life. - Adrenergic blockers, such as propranolol, may help the LVOT obstruction, but treatment is usually not necessary. Digitalis and other inotropic agents are contraindicated because they may worsen the obstruction. 3. If the LV is dilated with decreased LV contractility, the usual anticongestive measures (e.g., digoxin,diuretics) are indicated.

Transient Hypertrophic Cardiomyopathy in Neonates Recently, transient HCM in neonates who had perinatal injury with acute fetal distress has been described. Initially, echo studies showed abnormal LV systolic and diastolic function but the LV wall thickness was normal. The hypertrophy of the LV occurred between days 2 and 7 and affected initially the interventricular septum and later the LV posterior wall, but it disappeared in all cases between 1 and 5 months of life. Acute fetal distress with myocardial ischemia is believed to have caused the hypertrophy. The prognosis of this type of HCM is good, in contrast to that of other primitive HCM occurring in neonates ( Vaillant et al, 1997).

THANK YOU