At the Crossroads

Jennifer Ann L. Cantre, MD

Philippine Heart Center

General Data

• K D

• 22 female

• IT student

• Binangonan, Rizal

• Non-smoker, non-alcoholic beverage drinker

• Intermittent cyanosis

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

• 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

• 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

• April 2011

• Consulted at PHC OPD

• Work-up was done

• Advised surgery

• June 2011

• Patient got pregnant

• Referred to PCMC for prenatal check-up

• No progression of symptoms

• 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

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

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

Family History

• (+) maternal first cousin with CHD

• (-) Hypertension

• (-) diabetes mellitus

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

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

• 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

Impression

• Etiologic: Cyanotic congenital heart disease

• Anatomic: VSD, PS, TR

• Physiologic: SR

• Functional: NYHA I

Atrial rate: 65 bpm Ventricular Rate: 65 bpm PR = 0.16 QRS = 0.16 QT = 0.44

Sinus rhythm RVH CRBBB

Chest X-ray

2D-ECHO

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

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

RIGHT ATRIUM - Receives IVC

and Eustachian

valve

- Broad- based

and trabecular

appendage

LEFT ATRIUM - Receives

pulmonary veins

(usually)

- Long and finger-

like appendage

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

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

LVEDD 2.8

LVESD 1.9

IVSD 0.8

IVSS 1.0

PWD 0.5

PWS 1.0

LVMI 33

LV RWT 0.36

EF (M mode)

63 %

RVOT 1.5 cm

PV annulus 1.3 cm

MPA 1.3 cm

RVOT 68 mmHg

AT PV 72 mmHg

After PV 40 mm Hg

EF Simpson’s 59%

LAVI 16 ml/m2

RVFAC 54 %

TAPSE 1.9 cm

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

Hemodynamic Study

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

Hemodynamic Study

• Congenital Heart Disease, Double Outlet Right Ventricle

• Valvar and Infundibular Pulmonic Stenosis

• Patent Foramen Ovale

DISCUSSION

• 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.

• 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.

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

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

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

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

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

Donald J. Hagler MD. DORV and DOLV. Moss and Adams’ Heart Disease in Infants, Children and Adolescents.. Google books. 2012

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

INTERVENTIONS

M. A. Gatzoulis MD. et al. Diagnosis and Management of Adult Congenital Heart Disease. 2nd ed. 2011.

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

THANK YOU!

Surgical Management

patch closure of VSD

RV outflow MPA outflow patch (transannular patch)