Mitral Regurgitation

Etiology and pathophysiology

 Mitral regurgitation may result from disorders of the valve leaflets themselves or from any of
the surrounding structures that comprise the mitral apparatus.
 The leading cause of MR is rheumatic heart disease and less common conditions include mitral
annular calcification and congenital anomalies such as cleft MVs; other rare causes of MR are
endomyocardial fibrosis, carcinoid disease with right-to-left shunting, systemic lupus
erythematosus, and diet-drug toxicity.
 The second leading cause of MR in developed countries is “functional” MR, which results from
dilatation of the MV annulus or from myocardial infarction. In particular, infarctions involving
the inferolateral and the posteromedial papillary muscle produce tethering of the mitral leaflets
that prohibits normal coaptation, leading to “functional” MR even though the valve leaflets
themselves are normal

 Patients who develop acute severe MR  symptomatic heart failure  because their
ventricles are ill prepared to accept the sudden increase in volume load  if the patient
survives the acute episode? or has slowly progressive worsening? of MR  the left
ventricle is able to develop compensatory changes  Symptoms are therefore either
absent or slowly progressive over many years.

 The adaptive changes of the ventricle to the volume overload  LV dilatation and
eccentric hypertrophy. The left atrium also enlarges, thus allowing accommodation of the
regurgitant volume at a lower pressure.29

 Although patients with compensated chronic MR  remain asymptomatic for many

years

 Decompensation may eventually develop if the regurgitation is sufficiently severe  The

LV ejection fraction in chronic MR > normal  because of the increase in preload and
the afterload-reducing effect of ejection into the low-impedance left atrium  Therefore,
LV ejection fraction can be misleading as a measure of contractile function in this
disorder. Advanced myocardial dysfunction may occur while LV ejection fraction is still
well within the normal range. Thus, outcome after MV surgery is poorer in patients with a
preoperative ejection fraction of less than 60% than in those with higher ejection
fractions

Physical examinations
 According to the degree of decompensation.

 The carotid upstroke is sharp in patients with compensated MR, but the volume of the
carotid pulse is reduced in the presence of advanced heart failure.

 The apical impulse is usually brisk(cepat) and hyperdynamic; in those with severe
MR it may be enlarged and displaced laterally.

 The S1 is usually soft, and a widely split, S2 is common. A diastolic rumble and
S3 may be present and do not necessarily indicate LV dysfunction.

 The systolic murmur of MR varies according to the etiology of the regurgitation.

 The murmur is usually heard best at the apex in the left lateral decubitus position.

 With severe degenerative MR, the murmur is holosystolic, radiating into the axilla.

 Early systolic murmurs are typical of acute MR.

 Late systolic murmurs are typical of MV prolapse or papillary muscle dysfunction.

Diagnostic tests
Chest Radiography.

Cardiomegaly due to LV and left atrial enlargement is common in patients with chronic MR.
Kerley B lines and interstitial edema can be seen in patients with acute MR or progressive LV
failure.

Electrocardiography. Left atrial enlargement and atrial fibrillation are the most common ECG
findings in patients with MR. Left ventricular enlargement is noted in approximately one-third of
patients, and RV hypertrophy is observed in 15%.

Exercise Testing. Exercise testing is useful in determining functional capacity, particularly

when symptoms are unclear. Measurement of MR severity and pulmonary artery systolic
pressure before and after exercise using Doppler echocardiography can provide additional useful
information, especially if surgical intervention is being contemplated. This technique is
especially useful in symptomatic patients in whom there is a differentation between symptoms
and resting measures of LV function and pulmonary artery pressure.

Cardiac Catheterization. Cardiac catheterization is generally performed to assess the

hemodynamic severity of MR when noninvasive testing is inconclusive or a discrepancy exists
between clinical and noninvasive findings. Coronary angiography is indicated for patients who
are planning to undergo surgery and are at risk of CAD

Treatment
  Patients with mild MR and an otherwise normal heart may be followed up with annual
clinical examinations, undergoing echocardiography only if their clinical status changes
(eg, the intensity of the murmur changes)

 Patients with moderate to severe MR, clinical examination and echocardiography should
be performed yearly or sooner if symptoms develop.

 In view of the loading conditions  enhance LV shortening in severe MR, LV systolic

dysfunction in severe MR is defined as an ejection fraction of 60% or less or an end-
systolic dimension of 40 mm or greater.2 Such conditions should prompt surgical referral.

 Asymptomatic patients with severe MR  surgical correction, especially if the valve can
be repaired, after discussions regarding the benefit of early referral for surgery.  If such
patients decline surgery  clinical examinations and echocardiography every 6 to 12
months and immediate surgery if they develop symptoms, atrial fibrillation, pulmonary
hypertension, or LV systolic dysfunction. Recent data have shown that this “watchful
waiting” approach does not adversely affect survival as long as patients are carefully
monitored

LO 2 cogenital heart disease

Acyanotic Congenital Heart Disease : The Left-to-Right Shunt Lesions

Atrial septal defect
 Atrial septal defects (ASDs) can occur in any portion of the atrial septum (secundum,
primum, or sinus venosus), depending on which embryonic septal structure has
failed to develop normally.
 The majority of cases of ASD are sporadic; autosomal dominant inheritance does
occur as part of the Holt-Oram syndrome (hypoplastic or absent radii, 1st-degree
heart block, ASD) or in families with secundum ASD and heart block. Sering tjd pd
daerah fossa ovalis (tipe ostium sekundum)
 Jarang pd tipe sinus venosus (dibawah masuknya SVC ke RA)
 tekanan atrial atau vol. atrial kiri  foramen ovale membesar  darah dari LA
ke RA karena tekanan
 Pirau kanan ke kiri jika tekanan RA > LA
 Saat diastole: tekanan RA = LA
 Lebih sering 3x pd wanita
 Life span rerata pasien dg ASD tdk diobati: mid 50s

PF, PP dan tatalaksana

 • Kadang manifestasi sianosis pd minggu pertama (transient)

• Penonjolan prcordial  pembesaran RV

• Auskultasi: murmur, fixed splitting pd bunyi jantung 2 (indikasi komunikasi antar atrial)

• ECG

• Chest X-Ray

• Echocardiogram

• Penutupan surgical: bypass kardiopulmonal (pericardiotomy)

• Catheter-delivered devices: metal berbentuk payung

Coarctation of aorta

• Menyempitnya aorta descendens (juxtaductal: opposite site of ductus arteriosus)

• 2 jenis:

– Konstriksi aorta sendiri

– Hipoplasia arcus aorta & proximal aorta descendens  mengakibatkan gagal

jantung pd bayi

• Pd anak2 biasanya tdk jelas & lokasi pd distal dari asal arteri subclavian

• Obstruksi mekanik pd aliran darah dari LV  tekanan proksimal pd coarctation ,

setelah obstruksi tekanan normal atau   Δtekanan = fitur diagnostik! 
pembentukan arteri kolateral  vol. aliran darah   dilatasi pembuluh

• Hipertrofi LV karena tekanan sistolik  pd proximal coarctation

• Sering asimptomatik pd anak2 dg CA

• 10%  gagal jantung kongestif pd neonatal atau bayi

• Anak2 jarang tjd gagal jantung, namun menimbulkan:

– Sakit kepala berkaitan dg hipertensi pd porsi tubuh atas

– Nyeri dada (chest pain)

 • Predominan pd laki2 & turner syndrome

• PF:

– pertumbuhan normal, akrosianosis yg tdk berat

– Adanya bintik pd kulit karena edema pulmonal & perfusi yg buruk

– Δtekanan ekstremitas atas & bawah  palpasi radial & femoral beda. Radial 20
mmHg > femoral  indikator diagnostik!

– Pulsasi kencang pd radius bayi

– Murmur pd punggung blkg dkt tulang belakang

• PP:

– ECG

– Chest X-Ray: penonjolan aorta ascendens

– Terapi: operasi (end-end anastomoses) atau dilatasi balon (kateterisasi) u/

obstruksinya

Vetricular septum defect

• VSD (Ventricular Septal Defect)

– PBJ paling sering terdiagnosa

– VSD (isolated) biasanya pd bagian membranosa septum ventricularis

– Terkadang pd bagian Musculus septum ventricularis

• Multipel defek muscular: SWISS CHEESE TYPE

– Perpindahan darah antara ventrikel

– VSD baru muncul/terlihat pd bayi di usia 6-8 mgg

• Pd neonatus, Resistensi vaskuler pulmonal tinggi > resistensi vaskuler

sistemik krn dinding arteri pulmonalis tebal pd janin  output RV 
arteri pulmonalis, sebagian besar mengalir ke aorta mll ductus arteriosus,
hanya sebagian kecil yg masuk ke paru2 beresistensi tinggi & gasless
 • Setelah lahir, paru mengembang dg udara  PVR turun  plasenta
terputus dr sirkuit sistemiknya  resistensi vaskuler sistemik meningkat
2x  arteri pulmonal tipis & lumen lebar  left  right shunt

Pemeriksaan fisik

• Auskultasi: terdapat murmur

• Kardiomegali  apeks lebih lateral inferior atau penonjonal prekordial

• Takipnea

• Pertambahan berat badan yg lambat

• Sulit makan

• Defek ukuran besar  memicu gagal jantung pd usia 2-3 bln

PP dan tatalaksana

• ECG

• Chest X-Ray

• Echocardiography

• Cardiac Catheterization

– Mengidentifikasi koeksistensi anomali jantung lain, lokalisasi tempat VSD,

indikasi VSD multipel & gagal jantung, hemodinamik

• Operatif (korektif)

– Bypass kardiopulmo

– Patch of Darcon atau pericardium u/ menutup VSD

– Banding of pulmonary artery  meningkatkan PVR

PATENT DUCTUS ARTERIOSUS

• Komunikasi fetal antara aorta & truncus pulmonalis

• Biasanya tertutup pd hari ke 4 kehidupan

 • Dipengaruhi tensi O2 & penarikan prostaglandin endogen  penutupan DA

• Lebih sering ditemukan pd wanita, prematur & down’s syndrome

• Pd janin, duktus besar, PVR > SVR  darah mengalir dr kanan  kiri

• Setelah lahir, duktus paten, darah mengalir dari aorta  arteri pulmonalis

– PVR < SVR  darah dari kiri  kanan

• Jika aliran darah pulmonal terlalu besar  gagal jantung krn overload pd LV

Tanda gejala dan PF

• Kebanyakan pasien asimptomatis, hanya tdpt murmur. Jika simptomatik: mengalami

infeksi respirasi sering & mudah lelah

• PF:

– MACHINERY MURMUR

– Cyanosis pd ekstremitas bawah

Tatalaksana

• Prostaglandin Synthase Inhibitor

– Indomethacin, Ibuprofen

– Pd bayi prematur penutupan setelah pemberian oral atau IV 3 dosis

indomethacin (tiap 12 jam) atau ibuprofen (tiap 24 jam)

– KI thd insufisiensi renal & trombositopenia

• Pd dewasa, anak, bayi > 2 mgg tdk sukses dg obat

– Pemotongan & ligasi duktus

– Transkateter dg spring wire coils dilapisi benang2 Darconn (Gianturco coils)

Coarctation of Aorta

• Menyempitnya aorta descendens (juxtaductal: opposite site of ductus arteriosus)

• 2 jenis:

– Konstriksi aorta sendiri

– Hipoplasia arcus aorta & proximal aorta descendens  mengakibatkan gagal

jantung pd bayi

• Pd anak2 biasanya tdk jelas & lokasi pd distal dari asal arteri subclavian

• Obstruksi mekanik pd aliran darah dari LV  tekanan proksimal pd coarctation ,

setelah obstruksi tekanan normal atau   Δtekanan = fitur diagnostik! 
pembentukan arteri kolateral  vol. aliran darah   dilatasi pembuluh

• Hipertrofi LV karena tekanan sistolik  pd proximal coarctation

• Sering asimptomatik pd anak2 dg CA

• 10%  gagal jantung kongestif pd neonatal atau bayi

• Anak2 jarang tjd gagal jantung, namun menimbulkan:

– Sakit kepala berkaitan dg hipertensi pd porsi tubuh atas

– Nyeri dada (chest pain)

• Predominan pd laki2 & turner syndrome

• PF:

– pertumbuhan normal, akrosianosis yg tdk berat

– Adanya bintik pd kulit karena edema pulmonal & perfusi yg buruk

– Δtekanan ekstremitas atas & bawah  palpasi radial & femoral beda. Radial 20
mmHg > femoral  indikator diagnostik!

– Pulsasi kencang pd radius bayi

– Murmur pd punggung blkg dkt tulang belakang

• PP:

– ECG
 – Chest X-Ray: penonjolan aorta ascendens

– Terapi: operasi (end-end anastomoses) atau dilatasi balon (kateterisasi) u/

obstruksinya

Tetralogy of fallot (ToF)

• Kondisi jantung yg paling diketahui menyebabkan sianosis

• 4 komponen:

– VSD

– Pulmonary stenosis

– Generally infundibular in location

– RVH

• Secara hemodinamik, TOF merupakan kombinasi dari 2 lesi:

– VSD besar

– Stenosis pulmonal berat

• Terkait dg nfundibulum  respon thd katekolamin & stimuli  sianosis

bervariasi tergantung emosi & exersice

• TOF + atresia katup pulmonal = pseudotruncus arteriosus

– Darah tdk mengalir lgsg dari RV  arteri pulmonal, maka output keseluruhan mll
aorta

• Anak sianotik pd tahun pertama, kadang ada yg muncul awal (periode neonatus)

• Sianosis berat = stenosis pulmonal berat & derajat aliran darah pulmonal 

• 3 karakteristik pasien dg TOF:

– Derajat sianosis & gejala bervariasi

– Hipersianotik atau Tetrad spells (jika tindakan operatif awal maka tdk
ditemukan)
 • Tetrad spell: dyspnea (sulit nafas), sianosis, agitasi (emotional
disturbances), sinkop (unconciousness)

– Squatting (gerakan menjongkok)  resistensi vaskuler sistemik pirau

kanan-kiri  darah balik vena sistemik  RV stroke volume & aliran darah
pulmonal 

• PF:

– Sianosis

– Clubbing

– Murmur krn stenosis pulmonal (semakin berat stenosis semakin halus murmur)

• PP

– Chest X-Ray: heart is boot-shaped, apex turned upward, pulmonary artery

concave, pembesaran aorta ascendens

• Tatalaksana:

– 100% O2 ( resistensi sistemik)

– Posisi knee-chest

– Morfin atau ultrashort beta blocker

• Beta adrenergik bloker (mis: propanolol)   kontraktilitas miokardial

 gejala reda

– Α-antagonist (phenylephrine)

– IV fluid dg injeksi bolus

– Operatif:

• Blalock- Taussig Shunt (anastomosis arteri subclavian dg cabang arteri

pulmonalis)

• Waterston shunt (komunikasi antara arteri pulmonal kanan & aorta

ascendens)

• Potts procedure (arteri pulmonal kiri & aorta descendens)