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At the Crossroads
Jennifer Ann L. Cantre, MD
Philippine Heart Center
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General Data
• K D
• 22 female
• IT student
• Binangonan, Rizal
• Non-smoker, non-alcoholic beverage drinker
• Intermittent cyanosis
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History of Present Illness
• At birth – (22 years ago)
• patient was noted to be cyanotic
• Brought to PHC at 3 months of age : Told to have “butas sa puso”
• Advised work up
• Became lost to follow up
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• Interim
• Patient would complain of on and off effort-related dyspnea and cyanosis, (especially when playing with other kids) relieved by rest
• No consultation was done
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• May 2010
• Patient got pregnant
• On her 3rd month AOG:
– (+) vaginal spotting
– (+) hemoptysis
– Admitted at Morong Hospital, Rizal x 3 days
– (+) spontaneous abortion
– (+) PTB treatment x 6 months
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• April 2011
• Consulted at PHC OPD
• Work-up was done
• Advised surgery
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• June 2011
• Patient got pregnant
• Referred to PCMC for prenatal check-up
• No progression of symptoms
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• January 27, 2012
• She delivered to a 7 month old baby boy via cesarean section (fetal distress) at PCMC
• Hospital course was unremarkable and she was discharged after 6 days
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Past Medical History
– Born to a 27 year old G5P5(5004) mother who allegedly had unremarkable pregnancy with no intake of any medication nor infection
– Home delivery via NSD assisted by a midwife
– (+) cyanosis on crying
– (-) of frequent URTI nor hospitalizations
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OB Gyne History
– Menarch: 15 years old
– Regular monthly menstruation 3-6 days with occasional dysmenorrhea
– G2P1(0110)
• 2010 – 3mos spontaneous abortion
• 2012 – 7 mos , baby with multiple congenital anomalies -- > expired
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Family History
• (+) maternal first cousin with CHD
• (-) Hypertension
• (-) diabetes mellitus
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Review of Systems
• General: no weight loss, no anorexia
• HEENT: (+) Coryza
• Chest & Lungs: (+) Cough
• CVS: (-) chest pain
• GIT:(-) abdominal pain, no changes in bowel habits
• GUT: (-) dysuria
• Musculoskeletal: (-) leg swelling
• Neurologic: (+) headache
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Physical Examination
• Conscious, coherent, not in cardiorespiratory distress
• BP= 90/60mmHg RUE 100/60mmHg LUE 100/60mmHg RLE 100/70mmHg LLE
• HR= 85 bpm RR= 19 cpm • Wt= 35 kg Ht= 154 cm BMI=16 kg/m2 • O2 sat = 92-93% at room air
• Pink palpebral conjunctivae, anicteric sclerae, no nasoaural discharges, pink oral mucosa, no tonsillopharyngeal congestion
• No neck vein distention, no cervical lymphadenopathies
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• Equal chest expansion, spine deviated to the right, vesicular breath sounds
• Dynamic precordium, PMI 5th L ICS LMCL, (+) thrill 3rd ICS LPSB, (+) RV heave, normal S1, Single S2, (+) 3/6 SEM 2nd ICS LPSB, (+) 4/6 holosystolic murmur 3rd ICS LPSB, (+) 3/6 systolic murmur 4th ICS LPSB with (+) Carvallo’s sign
• Flat, (+) incisional CS scar, normoactive bowels sounds, no organomegaly, soft, nontender
• Full pulses, no edema, (+) clubbing of finger and toenails
(- ) cyanosis
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Impression
• Etiologic: Cyanotic congenital heart disease
• Anatomic: VSD, PS, TR
• Physiologic: SR
• Functional: NYHA I
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Atrial rate: 65 bpm Ventricular Rate: 65 bpm PR = 0.16 QRS = 0.16 QT = 0.44
Sinus rhythm RVH CRBBB
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Chest X-ray
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2D-ECHO
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Sequential Segmental Approach
1. Positioning of the heart within the chest
2. Determination of atrial and abdominal situs
3. Determination of atrioventricular relationship
4. Determination of ventriculo arterial relationship
5. Identification of shunts and valvular lesions
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IVC - Lies to the RIGHT of the spine - Collapsible - BLUE on color flow
AORTA - Lies to the LEFT of the spine - Larger, more rounded and more pulsatile - RED on color flow
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RIGHT ATRIUM - Receives IVC
and Eustachian
valve
- Broad- based
and trabecular
appendage
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LEFT ATRIUM - Receives
pulmonary veins
(usually)
- Long and finger-
like appendage
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RIGHT VENTRICLE -Trabeculated
endocardial
surface
-3 papillary
muscles
-chordae insert
into septum
-Infundibular
muscle band
- Moderator band
- triangular cavity
shape
-TV - apical
LEFT VENTRICLE -Smooth endocardial surface -2 papillary muscles -Ellipsoid geometry - MV - basal
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LVOT 2.4 cm
Ao Annulus 2.3 cm
Ao SOV 3.3 cm
Ao ST Junction 2.3 cm
Ao Ascending 2.8 cm
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LVEDD 2.8
LVESD 1.9
IVSD 0.8
IVSS 1.0
PWD 0.5
PWS 1.0
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LVMI 33
LV RWT 0.36
EF (M mode)
63 %
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RVOT 1.5 cm
PV annulus 1.3 cm
MPA 1.3 cm
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RVOT 68 mmHg
AT PV 72 mmHg
After PV 40 mm Hg
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EF Simpson’s 59%
LAVI 16 ml/m2
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RVFAC 54 %
TAPSE 1.9 cm
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2DED
• Congenital Heart Disease, Double Outlet Right Ventricle • Large subaortic VSD with bidirectional shunt • Pulmonic stenosis, infundibulovalvar with peak gradient of
68mmHg at the RVOT and 72mmHg across the PV • Thickened and redundant anterior mitral valve with trivial
mitral regurgitation • Anterior and posterior tricuspid valve prolapse with severe
tricuspid regurgitation. Dilated TV annulus • Thickened aortic valve • Small and compressed LV with adequate contractility and
systolic function • Dilated RV with thickened walls with signs of pressure and
volume overload • Dilated RA and coronary sinus • Hypoplastic main pulmonary artery
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Hemodynamic Study
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91
67.8
75.6
57.8
64
70.3
55.7
59.3
67.8
79.6
75.9
91.3
91.4 97.5
94.7 9
25/10
57/8 5
116/10
107/84
94/9
0XYGEN SATURATION
PRESSURES
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Hemodynamic Study
• Congenital Heart Disease, Double Outlet Right Ventricle
• Valvar and Infundibular Pulmonic Stenosis
• Patent Foramen Ovale
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DISCUSSION
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• The severity or lack of pulmonary stenosis largely determines the spectrum of symptoms and the patient's age at the time of clinical presentation.
• In general, most patients present during the neonatal period.
• Patients with severe pulmonary stenosis have cyanosis, and those with uncontrolled pulmonary blood flow present with congestive heart failure.
Double Outlet Right Ventricle Surgery Abdullah A Alghamdi, MD, FRCSC; Chief Editor: John Kupferschmid, MD. Medscape.
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• incidence of double outlet right ventricle is 0.09 cases per 1000 live births.
• comprises about 1-1.5% of all congenital heart disease
Double Outlet Right Ventricle Surgery Abdullah A Alghamdi, MD, FRCSC; Chief Editor: John Kupferschmid, MD. Medscape.
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Embryology
• Embryonic arrest in the normal rotation of the junction of the outflow tract and great arteries
• The aorta is usually positioned to the right of and anterior to the pulmonary trunk or the aortopulmonary relations are side by side with the aorta to the right
• Each great artery arises above an infundibulum (conus), preventing fibrous continuity with AV valve tissue
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DORV WITH SUBAORTIC VSD - blood from the LV flows through the VSD to the aorta and blood from the RV flows to the aorta or PA, yielding physiology similar to Tetralogy of Fallot
Double Outlet Right Ventricle Surgery Abdullah A Alghamdi, MD, FRCSC; Chief Editor: John Kupferschmid, MD. Medscape
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DORV WITH SUBPULMONARY VSD - Taussig-Bing syndrome
- blood from the LV flows through the VSD to the pulmonary artery and blood from the RV flows mainly to the aorta, yielding physiology similar to Transposition of the Great Art
Double Outlet Right Ventricle Surgery Abdullah A Alghamdi, MD, FRCSC; Chief Editor: John Kupferschmid, MD. Medscape
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DORV WITH DOUBLY COMMITTED VSD - The infundibular septum is absent leaving both aortic and pulmonary valves related to the VSD. Clinical features depend on the presence or absence of pulmonary stenosis.
Double Outlet Right Ventricle Surgery Abdullah A Alghamdi, MD, FRCSC; Chief Editor: John Kupferschmid, MD. Medscape
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DORV WITH NONCOMMITTED VSD - blood from both ventricles is substantially mixed in the RV, yielding physiology that resembles a large VSD
Double Outlet Right Ventricle Surgery Abdullah A Alghamdi, MD, FRCSC; Chief Editor: John Kupferschmid, MD. Medscape
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Donald J. Hagler MD. DORV and DOLV. Moss and Adams’ Heart Disease in Infants, Children and Adolescents.. Google books. 2012
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Position of the VSD and Oxygen Saturation
• When the VSD was subaortic, the saturation varied
• In 60% of patients with subaortic VSD, the systemic arterial saturation was greater than the pulmonary arterial saturation and 40% had pulmonary arterial saturation greater than systemic arterial saturation.
• The presence or absence of PS or pulmonary vascular obstruction had no consistent effect on systemic or pulmonary arterial saturation
Donald J. Hagler MD. DORV and DOLV. Moss and Adams’ Heart Disease in Infants, Children and Adolescents.. Google books. 2012
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INTERVENTIONS
M. A. Gatzoulis MD. et al. Diagnosis and Management of Adult Congenital Heart Disease. 2nd ed. 2011.
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Surgical results in patients with double outlet right ventricle: a 20-year experience
John W. Brown, MD*a,b, Mark Ruzmetov, MDa,b, Yuji Okada, MDa,b, Palaniswamy Vijay, PhD, MPHa,b, Mark W. Turrentine, MDa,b
• Methods. Patients (n = 124; June 1980 to January 2000; age range, 7 days to 16 years; mean, 2.8 years) who underwent repair of double outlet right ventricle.
• Results. Four types of definitive repairs were performed: (1) intraventricular tunnel repair with a baffle from the left ventricle to the aorta (n = 53); (2) use of a valved or nonvalved conduit (n = 20); (3) arterial switch operation with a patch committing the left ventricle to the neo-aorta (n = 16); and (4) cavopulmonary shunt and Fontan procedures (n = 33)
• Conclusions. Survival was high for all patients with double outlet right ventricle undergoing intraventricular tunnel repair, arterial switch operation, and repair with a conduit or a modified Fontan procedure.
Ann Thorac Surg 2001;72:1630-1635
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THANK YOU!
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Surgical Management
patch closure of VSD
RV outflow MPA outflow patch (transannular patch)