ATRIAL SEPTAL DEFECT
PRESENTED BY :-
DR KUNWAR SIDHARTH SAURABH
DEFINITION
AN ATRIAL SEPTAL DEFECT IS A HOLE OF
VARIABLE SIZE IN THE ATRIAL SEPTUM.
A PATENT FORAMEN OVALE THAT
IS FUNCTIONALLY CLOSED BY
OVERLAPPING OF LIMBIC
TISSUE SUPERIORLY AND THE VALVE OF
FOSSA OVALIS INFERIORLY IS EXCLUDED.
DEVELOPMENT
PAPVC
• PARTIAL ANAMOLOUS VENOUS CONNECTION
IS A CONDITION IN WHICH SOME BUT NOT ALL
PULMONARY VEINS CONNECT TO THE RIGHT
ATRIUM OR ITS TRIBUTARIES RATHER THAN
LEFT ATRIUM.
FOSSA OVALIS
DEFECT/
OS-ASD/
FORAMEN
OVALE TYPE
POSTERIOR DEFECT
SINUS VENOSUS
DEFECT/
SUBCAVAL DEFECT/
SUPERIOR VENA
CAVAL DEFECT
SINUS VENOSUS DEFECT
LOCATED IMMEDIATELY BENEATH THE ORIFICE OF SVC.
USUALLY ASSOCIATED WITH PAPVC OF RSPV.
THE LOWER MARGIN OF THE DEFECT IS SHARPNED WHEREAS THE UPPER MARGIN IS
DEVOID OF SEPTUM BEING CONTINUOS WITH THE POSTERIOR SVC WALL.
THE SVC USUALLY OVER-RIDES THE ATRIAL SEPTUM ON TO THE LEFT ATRIUM.
CORONARY SINUS DEFECT/
UNROOFED CORONARY SINUS SYNDROME
OSTIUM PRIMUM DEFECT/ AV CANAL DEFECT/ AV SEPTAL DEFECT
ASD OCCURS ANTERIOR TO THE FOSSA OVALIS WHEN THE AV SEPTUM IS ABSENT.
TYPES OF PAPVC
SINUS VENOSUS MALFORMATION
• MOST COMMON TYPE.
• PAPVC CO-EXISTS WITH SUPERIOR CAVAL ASD.
• RIGHT UPPER AND MIDDLE LOBE PULMONARY VEINS CONNECT TO
LOW SVC OR AVC – RIGHT ATRIAL JUNCTION - M/C ARRANGEMENT.
• SVC MAY BE SMALL IN SIZE IF LSVC PRESENT.
• OVER RIDING OF SVC, IF SEVERE, MAY PRODUCE CYANOSIS.
SINUS VENOSUS
MALFORMATION
RIGHT SUPERIOR PULMONARY VEIN TO SVC
RIGHT PULMONARY VEINS TO RIGHT ATRIUM
RIGHT
PULMONARY
VEINS TO IVC
(SCIMITAR
SYNDROME)
• ENTRANCE INTO THE IVC JUST SUPERIOR TO
THE HEPATIC VEINS.
• ISOLATED MALFORMATION IN RARE CASES
• USUALLY ASSOCIATED WITH THE
MALFORMATION OF THE RIGHT LUNG MOSTLY
RIGHT LUNG HYPOPLASIA.
• BLOOD SYPPLY TO HYPOLASTIC LUNG COMES
FROM BRANCHES OF ABDOMINAL AORTA.
• RIGHT LOWER LOBE BRONCHOPULMONARY
SEQUESTRATION MAY CO-EXIST.
SCIMITAR SYNDROME
LEFT
PULMONARY
VENOUS
CONNECTION
MAY CONNECT TO LEFT
BRACHIOCEPHALIC VEIN BY
MEANS OF ANAMALOUS
VERTICAL VEIN.
ASSOCIATED
CARDIAC
CONDITIONS
CARDIAC
CHAMBERS IN
ASD
RA IS GREATLY ENLARGED AND THICK
WALLED.
RV DIASTOLIC SIZE IS INCREASED BECAUSE
OF VOLUME OVERLOAD.
NORMAL RV DIMENSIONS – 0.6 TO 1.4 cm .
M2
IN LARGE LEFT TO RIGHT SHUNTS ITS
AVERAGE 2.66 cm.m2.
THE CARDIAC APEX IS OFTEN FORMED BY RV.
LV SIZE IS NORMAL BUT DYNAMIC
ABNORMALITIES MAY BE PRESENT.
MITRAL VALVE
AND ASD
• INCREASED PREVALENCE WITH AGE
• ALTERATION IN LV CONFIGURATION
RESULTING FROM THE SHIFT OF THE
VENTRICULAR SEPTUM.
MITRAL PROLAPSE -
• MITRAL PROLAPSE CAN LEAD TO MR.
• 5% CASES REQUIRE CORRECTION AT THE TIME
OF ASD CLOSURE.
MITRAL REGURGITATION -
• OCCUR OCCASIONALY
CLEFT MITRAL LEAFLETS -
HEATH EDWARDS CLASSIFICATION
LUNG AND PULMONARY VASCULATURE
• PULMONARY ARTERIES ARE CONSIDERABLY DILATED DUE TO
INCREASED BLOOD FLOW.
• PULMONARY HYPERTENSION DOES NOT DEVELOP UNTIL 3RD OR 4TH
DECADE.
• THIS CONTRASTS SHARPLY WITH VSD, IN WHICH PULMONARY
VASCULAR DISEASE ID PRESENT IN EARLY LIFE.
• IN ASD, IT IS MAINLY CAUSED BY SECONDARY THROMBOSIS IN
THE DILATED PULMONARY ARTERY BRANCHES WITH CHANGES IN
INTIMA AND MEDIA PLAYING A MINOR ROLE.
EPIDEMIOLOGY
• MOST COMMON ADULT CONGENITAL HEART
DISEASE
• 2-3:1 – FEMALE : MALE
• OCCURS 1 IN EVERY 1500 BIRTHS
• 6-10% OF ALL CHD
ASSOCIATED
SYNDROMES
• SPORADIC
• HOLT ORAM SYNDROME
• PATAU SYNDROME (TRISOMY 13)
• EDWARD SYNDROME (TRISOMY 18)
• DOWN SYNDROME
• TURNER SYNDROME
• WOLF HIRSCHORN SNDROME
RAGHIB SYNDROME
• ASD
• LSVC TO LT ATRIUM
• ABSENT CORONARY SINUS
PATHOPHYSIOLOGY
• SHUNT FLOW IS DETERMINED BY -
• SIZE OF DEFECT
• RELATIVE COMPLIANCE OF VENTRICLES
• PULMONARY VASCULAR RESISTANCE
• OBSTRUCTION TO VENTRICULAR OUTFLOW
TRACT
PATHOPHYSIOLOGY
RV TOLERATES EXTRA VOLUME
OVERLOAD QUITE WELL AS IT CAN
HANDLE LARGE VOLUME AT LOW
EJECTION PRESSURES.
FACTORS FAVOURING LEFT TO RIGHT
SHUNT -
RIGHT HEART IS
MORE
DISTENSIBLE
TV IS MORE
CAPACIOUS
RV IS THIN
WALLED
DECREASED
PVR INCREASES
RV STROKE
VOLUME
NATURAL
HISTORY
THE NATURAL HISTORY OF THE PERSONS BORN WITH
ASD IS NOT KNOWN PRECISELY.
IN 1970, CAMPBELL PUBLISHED THE MOST DETAILED
STUDY ON PATIENTS WITH ASD TREATED NON
SURGICALLY.
99.9 % NEONATES WITH LARGE ISOLATED ASD
REACH THEIR 1st YEAR OF LIFE
5-15% DIE IN THEIR THIRD DECADE WITH PHT AND
ES.
NO MORE THAN 25% DIE FROM THE DEFECT
MORTALITY
RATE
SPONTANEOUS
CLOSURE
FUNCTIONAL
STATUS
CHANGES IN
PULMONARY/SYSTEMIC
BLOOD FLOW
DECREASING LV COMPLIANCE
OVER AGE : INC Qp/Qs
ASD SIZE INCREASES WITH
AGE
DECREASE IN RV COMPLIANCE
WHEN PHT DEVELOPS : DEC
Qp/Qs
RIGHT
VENTRICULAR
FUNCTION
RVVO
INCREASED RV END
DIASTOLIC DIMENSIONS
RVVO WELL TOLERATED
THAN LVVO
EVENTUALLY RV FAILURE
OCCURS
LEFT
VENTRICULAR
FUNCTION
LOSS OF LV FUNCTIONAL
RESERVE
EXERCISE INTOLERANCE
SUB-NORMAL DIASTOLIC
DIMENSIONS
RESULT OF RVVO
ARRYTHMIAS
AFTER THIRD DECADE ,
SUPRAVENTRICULAR ARRYTHMIAS
COMPLICATE THE NATURAL HISTORY OF
PATIENTS WITH LARGE ASD AND RELATED
CONDITIONS IN INCREASING NUMBER
OVER TIME.
BEGINS AS PAROXYSMAL AF, GRADUALLY
BECOMES PERMANENT.
CLINICAL
FEATURES
OFTEN ASYMPTOMATIC UNTIL 3-4TH DECADE.
FATIGUE AND BREATHLESSNESS ON EXERTION OCCURS WITH
ADVANCING AGE
RECURRENT RESPIRATORY TRACT INFECTIONS
ARRYTHMIAS CAUSE PALPITAIONS
PARADOXICAL EMBOLUS
CHRONIC HEART FAILURE IN OLDER INDIVIDUALS
CYANOSIS – ATYPICAL PRESENTATION
Qp/Qs - >1.5
Physical signs
LEFT PARASTERNAL
HEAVE
WIDE FIXED S2
SYSTOLIC EJECTION
MURMUR
MID DIASTOLIC TV
FLOW MURMUR.
ECG
SINUS ARRYTHMIA
PROLONGED PR INTERVAL
INCOMPLETE RBBB
CROCHETAGE
CHEST X-RAY
& 2D ECHO
REFLECT LARGE QP/QS
RV ENLARGEMENT
RA ENLARGEMENT
PLETHORIC PULMONARY FIELDS
ECHO CAN GIVE DIRECT EVIDENCE OF ASD, RVVO, AND
CHAMBER DIMENSIONS, ALONG WITH THE DIRECTION
OF THE SHUNT
X RAY & 2D ECHO
CARDIAC
CATHETERISATION
• IN INFANTS AND IN ANY PATIENTS IN WHOM
NON INVASIVE TESTS SUGGEST PAPVC.
• FOR ASSESMENT OF OPERABILITY IN PATIENTS
WITH PHT.
• THE MOST RELIABLE CRITERIA OF
INOPERABILITY IS THE ABSOLUTE LEVEL
OF PULMONARY VASCULAR RESISTANCE
NORMALISED TO BSA (RpI).
• PRECISE CRITERIA NOT ESTABLISHED FOR ASD
AND CRITERIA ESTABLISHED FOR VSD IS
FOLLOWED.
• A RESTING RpI > 8 U MAY PRECLUDE COMPLETE OPERATIVE CLOSURE.
• IF ARTERIAL DESATURATION <97% EXISTS WHEN MEASURED BY PULSE
OXIMETER, CARDIAC CATHETERISATION IS INDICATED.
• PULOMNARY VASCULAR REACTIVITY IS ALSO TESTED.
• A VASODILATOR MUST PRODUCE SPO2 LEVELS TO NORMAL OR RpI <7
BEFORE PATIENT CAN BE CONSIDERED OPERABLE- BECAUSE ONLY
THEN IT CAN BE EXPECTED THAT PHT WILL REGRESS AFTER
CLOSURE. OTHERWISE DISEASE WILL CONTINUE TO PROGRESS.
• PROGRESSIVE PULMONARY VASCULAR DISEASE IS LESS WELL
TOLERATED WHEN THE ATRIAL SEPTUM IS INTACT BECAUSE THE
RIGHT SIDE OF THE HEART IS UNABLE TO DECOMPRESS
THROUGH RT--> LT SHUNT, THUS ASD CLOSURE UNDER SUCH
CIRCUMSTANCES CAUSES DECREASE IN LIFE EXPECTANCY.
INDICATIONS
FOR
OPERATION
ASD WITH RVVO
QP/QS> 1.8
SCIMITAR SYNDROME EVEN IF QP/QS <1.8 WITH
SEVERE HYPOPLASIA OF LEFT LUNG.
ISOLATED PAPVC -
• PART OF ONE LUNG WITH QP/QS <1.8 - NOT INDICATE
• PAPVC OF WHOLE LUNG – INDICATED
MOD MR TO BE ADDRESSED AT THE TIME OF
CLOSURE
CONTRAINDICATIONS
PULMONARY VASCULAR DISEASE > 10 WU/m2
NON REACTIVE PULMONARY VASCULAR BEDS.
IN SCIMITAR SYNDROME IN INFANTS – IT MAY
BE SUFFICIENT SIMPLY TO INTERRUPTTHE
ABBERANT ARTERIAL SUPPLY TO THE
HYPOPLASTIC LUNG AND LEAVE THA
ANAMOLOUS VENOUS CONNECTION INTACT.
SURGICAL
TECHNIQUES
REPAIR OF SINUS
VENOSUS
MALFORMATION
WARDEN PROCEDURE
WARDEN
CONTD..
REPAIR OF
ANAMOLUS
CONNECTION
OF RSPV AND
RIPV TO RA
SCIMITAR
SYNDROME
SCIMITAR
SYNDROME
FOR PATIENTS
WITH HIGH
PVR
OTHER
SURGICAL
OPTIONS
MICS
RIGHT THORACOTOMY
ROBOTIC
POST OP
CONVALSENCE MOSTLY
UNEVENTFUL
IF MR SUSPECTED – REOPERATE
INCIDENCE OF PULMONARY AND
SYSTEMIC EMBOLISATION –
ANTICOAGULATION (WARF) FOR 8-
12 WEEKS IN OLD PATIENTS
CHOICE OF
PATCH
• WHEN REGURGITATION JET MAY
STRIKE THE PATCH
• WHEN PERICARDIUM FROMS THE
WALL OF BAFFLE
• DELICATE AREA
PERICARDIUM -
PTFE
DACRON
THANKS

Asd