CONGENITAL HEART DISEASES

Practical approach in children with heart murmurMIHAI CRAIU MD PhDIMCC

NORMAL HEART SOUNDS

NORMAL HEART SOUNDS

A normal heartbeat makes two sounds like "lubb-dupp" (sometimes described as tumm-taah"), which are the sounds of heart valves closing .

NORMAL HEART SOUNDS

The normal sounds are: the first heart sound (S1) produced by the closing of the AV valves and second heart sound (S2), produced by the closing of the semilunar valves.

NORMAL HEART

CARDIAC CYCLE

HEART MURMURS

Heart murmurs are abnormal sounds during cardiac cycle generated by turbulent blood in or near the heart.

HEART MURMURS

Murmurs may be physiological (benign) or pathological (abnormal).

HEART MURMURSAbnormal murmurs can be VALVULAR caused by:stenosis restricting the opening of a heart valve, or with valvular insufficiency which allows backflow of blood when the incompetent valve closes

MITRAL VALVE INSUFFICIENCY

HEART MURMURSAbnormal murmurs can be NON-VALVULAR caused by:Septal defectsASD, VSDEndocardial cushion defectPatent ductus arteriosusOut-flow tract stenosisVascular stenosis (Ao, AP)

CONGENITAL HEART DISEASEThere are three different hemodinamic CHDs with abnormal murmurs

Left to right shunt (NO cyanosis)Right to left shunt (WITH cyanosis)No shunt (vascular)

CHD with L/R shuntNO CYANOSIS!!

Most frequent group of CHDs

Most of them are completely curable with cath-lab procedures or surgery

CHD with L/R shunt

ASD (atrial septal defect)

VSD (ventricular septal defect)

PDA (patent ductus arteriosus)

VSDVSDs are the most common congenital cardiac anomalies. They are found in 30-60% of all newborns with a congenital heart defect, or about 2-6 per 1000 births. Congenital VSDs are frequently associated with other congenital conditions, such as Down syndrome

VSD

A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart.

VSD

The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes.

BLOOD FLOWDuring ventricular contraction some of the blood from the left ventricle leaks into the right ventricle (SHUNT), passes through the lungs and reenters the left ventricle via the pulmonary veins and left atrium.

PATHOPHYSIOLOGYL/R SHUNTING produces:volume overload on the left ventricle (shunt + venous return from lungs)high right ventricular pressure and volume, causing PAH (pulmonary artery hypertension)

VSDIn severe cases, the pulmonary arterial pressure can reach levels that equal the systemic pressure. This reverses the shunt, so that blood then flows from the right ventricle into the left ventricle, resulting in cyanosis, as blood is by-passing the lungs for oxygenation (Eisenmenger syndrome = fixed PAH)

SIGNS & SIMPTOMSVSD is an acyanotic congenital heart defect Pansystolic (Holosystolic) murmur (depending upon the size of the defect) +/- palpable thrill (palpable turbulence of blood flow). An infant with a large VSD will fail to thrive and become sweaty, pale and tachypnoeic with feeds

LAB

ECG( PREDOM DE R mai mari decat S)

Chest Rx(cardiomegalie)

Ultrasound(confirma rx)

ECG of VSD

Rx in VSD

Echo of VSD

TREATMENTMost cases do not need treatment and heal at the first years of life. Treatment is either conservative or surgical. Smaller congenital VSDs often close on their own, as the heart grows, and may be treated conservatively.

SURGERYShould be done in selected cases:Failure of congestive cardiac failure to respond to medicationsVSD with pulmonic stenosisLarge VSD with pulmonary hypertensionVSD with aortic regurgitation

OCCLUDERS / AMPLATZER

CHD with CYANOSISFallot tetralogy (trilogy or pentalogy)TGATruncus arteriosusPersistent fetal circulationEndocardial cushion defect (AV def)Univentricular heart

TETRALOGY of FALLOTA congenital cardiac anomaly with cyanosis that consists of four defects: pulmonic stenosis, ventricular septal defect, malposition of the aorta so that it arises from the septal defect or the right ventricle (Overriding aorta), right ventricular hypertrophy.

TETRALOGY of FALLOT

Occurs in less than 1 in 2000 live births.

Tetralogy of Fallot is the most common cyanotic congenital heart defect.

TETRALOGY of FALLOT

SIGNS & SIMPTOMS

SQUATTING

LABCBCIncresed Hb levelPolycytemia( dam aspirina!!)Incresed platelet countSa nu aibe trombocitoza!!)

CHEST Rx in FALLOT

ECHO in FALLOT

TREATMENT

DIFFERENTIALS FOR FALLOT

Auscultare incepe de la apex( mitrala ) pana la baza!!

Incepem cu focarul mitralei( mai aproape de linia mediana, pana la mamelon)..Apoi fpcarul AO( dreapt spt intercostal 2).apo focarul PLTRICUSCPIDA IN REG XIFOIDULUI!!

IMENSA MAJ A ZGOMOTELOR DE LA TRICUSPIDA( REGUrgitare fiziologic)*Z1- cel mai SCURT!!....Z2- CEL MAI LUUNG!1

La o frecventa de 140/min nu stii care este zgomotul 1 sau 2.Cu cat este mai mci cu atat frecventa este ft mare iar zgomotele au aceeasi cadenta(distgingem prin tonalitate)*z1_= inchidere valve AV!!!Z2=.INCHIDERE VALVE SEMILUNARE!!(AORTO PULMONRE)

*Particularitati de curgere a sangelui!La Copil exista niste part de curgere a sangelui:Etapa de tranziyie de la circ fetala la cea postnatala!!La nn se intampla se intampla urm procese:Sangele care vine prin cele 2 vene cave se varsa in atriul drept.si nu conteaza in care parte a corpului se afla aatriul in care este vci.este denumit generic AD!!. Atriul venos este acela in care se varsa VCI!!!VD are o artera pulmonara mica care se duce inspre plamani!!.....are un tract de ejectie dn care pleaca artera pulmonara MICA SI CONSTRICTIE FT IMPORTANTA A ARTERIOLELOR PULMONARE PANA LA NASTERE!!

RESTUL se duce prin CANALUL ARTERIAL PATENT( ASIGURA UN SUNT DREAPTA STANGA)O SUNTARE DE LA ARTERA PULM LA CROSA AORETI!! FORAMEN OVALE ( INTRE AS-AD).CARE ARE O PERDELUTA LA STANGA INSPRE AS!!.Intre VD SI AD SE afla valva atrioventriculara!! O valva cu 3 cuspe!!.....

Mitrala are 4 foite in viata intrauterina!!

*La clamparea cordonului ombilical!!....creste brusc rezistenta arteriala!!, creste presiunea in artera pulmonara!! Si scade fluxul de sange prin canalul arterial!!!FORAMEN OVALE SE INCHIDE COMPET IN ZILE SAPTAMANI

O PARTE CU PRIMA RESP.Hb ESTE MAI BN SATURATA ASTA RELAXEAZA MUSC ARTERIOLE PULMONARE astfel se duce mult sange prin artera pulmonara, canalul arterial nu mai sunteaza, se inchide!!!( e ip cu saturatia Hb) astfel creste pres pe versantul stang al inimiii!!

*Murmurul sau suflul ( sunt fluxuri de sange care curg anormal)!!!......apar turbulente!!Murmurul poate fi: fiziologic!! ( minime intarzieri intr-un curs normal al dezvoltarii)!!

Patologice!! anormale!...produse de anomalii valvulare!!

*Sufluri valvulare cauzate de stenoza!!..... Sau de insuficienta valvulara!!*Anomalii murmur nonvalvulare!!:Defecte de sept: ASD.VSD!!ENDOCARDIAL CUSHION DEFECT(LIPSESC PERNITELE ENDOCARDIACE PE CARE SE INSERA VALVELE)!!! ATRIILE NU MAI SUNT SEPARATE DE VENTRICULI!

DUCT ARTERIAL PATENT!!STENOZE DE VASE DE EJECTIE!1STENOZA VASCULARA!!(AO AP)*Malformatiile cu sunt stanga_ dreapta ( fara cianoza!!)Malformatii dreapta spre stanga( cu cianoza!!!)

Fara sunt ( vascular!!)*Fara cianoza!! Defectul de sept interventricular!! Nu e cea mai frecventa!1

*2-6/1000 au DSV!! DSV ESTE ASOCIAT CU SINDROM DOWN!11

SINDR DOWN E CEA MAI FRECVENTA ANEUPLOIDIE 1/700 NASTERI!!!

ACESTI COPIIPOT AVEA DIVERSE GRADE DE DEVZOLTARE ALE ORGANELOR.FORME CU MOZAIC IN CARE DOAR UNELE ORGANE SUNT AFECTATE: Unii pacienti operati pe cord , se fac prelevari endomiocardiace!! ( au down doar la inima!!!) au trisomie la inima!

Gena DSCR1 resp de aceste malformatii cardiace!

*Ventriculul se def dupa valva de deasupra lui( daca are deasupra valva mitrala!!!)Aceasta anomalie a peretelui poate sa fie localizata in diverse zone!! DVS IN PLIN MUSCHI!.....FLUXUL DIN PARTEA STANGA CATRE DREAPTA!! (SUNT STANGA DREAPTA) FARA CIANOZA, CRESTE DEBITUL CARDIAC!DSV PERIMEMBRANOS.DACA SEPTUL MUSC E INTACTESTE MAI SUS SITUATIN TIMPUL contractiei oparte dn sange trece prin suntare si incarca circ pulmonara!!...suntul stanga streapta dn DSV produce supraincarcare de volum de partea dreapta!!. VD pompeaza cu presiuni mai mari!!! Si apare HTP. Ventriculul stang primeste mai mult sange!!

Astfel daca nu se mai aude suflul!!.....inseamna ca s-a dezvoltat Hipertensiune Pulmonara!! Decompensare ventricul drept!!

Sindrom Eisenmeger!!*Cand ventriculul drept ajunge sa pompeze ft mult sange presiunea se egalizazea cu cea sistemica.apare sindromul eisenmenger!! Ceea ce inseamna HTAP FIXA( MUSCHII ARTERIOLARI SUNT SPASTICI, FIXI)

*SEMNE SI SIMPTOME!: FARA CIANOZA..SUFLUL ESTE HOLOSISTOLIC!!! PRIN GAURI MICI AR TREBUI SA EXISTE SUFLURI ROMBOIDALE

PRIN GAURI MARI.SE AUDE SUFLU CONTINUU!!!

ACEST SUFLU SE AUDE: in plin stern! Spatiul 3-4 intercostal si se aude in spita de roata!! Iradiaza!!se aude interscapulovertebral!Se poate si palpain regiunea precordiala ( suflurile mari de grad 5/6)

Un copil cu un DVS LAARG .va avea falimentul cresterii.si va deveni transpirat!/ palid si tahipneeic!! ( are semne de congestie!!!) asem cu ICC

Rau este un suflu care se estompeaza si un pacient care devine ft cianotic!!

*Supraincarcare dreapta

r-uri mai mari decat s in precord drepte*Indicele cardio toracic..10kg!)

Operatie paleativa: anastomozaintre a. subclavie stanga se pune un tub de dacron intre subclavia stanga- a. pulm stanga!!!Sunt sistemico-pulmonar!!! ( pt a trimite putin oxigen catre plamani)se poate folosi cateva luni pt a mai creste copilul!!

Pt a creste arterle pulmonare *DD: transpozitia de mari vase!!!( SANGELE NU SE INTERSECTEAZA DELOC, IN viata IU nu are importanta acest lucru!!

Operatia de transpozarae presupune : inciziese taie originea coronarelor pt a repozitiona aorta si se reimplanteaza coronarele..si pulmonara la ventriculul omonim!!!!

Dupa varsta de 10 zile nu se mai reimplanteaza coronarele!!!! Operatia de mari vase se face imediat!!Are cianoz imediat dupa nastere!!!

Rx: seamana cu un OU!!.....

Operata in primele zile de viata!!ECO: vasele sunt in teava de pusca!!!( vasele sunt una langa alta!!

TRUNCHI ARTERIAL DE TIP 1.FT GREU DE CORECTAT!!PCA: TASNITURA E ABUR!!!

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