The normal twin, called the pump twin, drives blood through both fetuses. It is called reversed arterial perfusion because in the acardiac twin the blood flows in a reversed direction. TRAP sequence occurs in 1% of monochorionic twin pregnancies and in 1 in 35,000 pregnancies overall.

1. (Fetal Acardia in Twin Gestation)
MUKESH SAH
POST GRADUATE MEDICAL INTERN
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2. 06/05/2020 2

3. 
 A 26 year old G2P1(1001) was admitted at our institution for the first
time due to labor pains with a diagnosis of G2P1(1001) Pregnancy uterine
33 6/7wks AOG, Cephalic in preterm labor, Twin gestation, Twin A fetal
hydronephrosis, Twin B Acardia with confirmation of Doppler ultrasound
which was done at 3rd trimester.
I. Abstract
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4. 
 On admission, no pallor was noted and the patient had stable vital signs
with on and off hypogastric tenderness. The cervix is dilated to 3cm, beginning
effacement, cephalic, station -2, intact bag of water with show. A decision was
made by the admitting service that expectant management with uterine
contraction and cervical dilatation monitoring was in order.
I. Abstract
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5. 
 During the course of labor monitoring, patient condition
progress to active labor after 24hours with a cervical
dilatation of 6cm, 60% effaced, station -2, cephalic with show.
She subsequently underwent emergency low transverse
cesarean section delivering a set of twins, twin a live baby
girl with congenital hydronephrosis, twin B was acardiac,
acephalous baby girl.
 In this case, the acardiac baby had 100% mortality and the
pump twin eventually died after few hours of life due to
cardiac failure as theorized.
I. Abstract
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6. 
 Acardiac twin pregnancy is uncommon and rare, hence presenting a
therapeutic challenge worthy of review. The management of this case has been
successful in delivering both the twin via low transverse cesarean section but the
dilemma was the fetal outcome due to lack of fetal surveillance and invasive
treatment in improving perinatal outcome.
I. Abstract
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8. 
Acardiac twin occurs only in monozygotic twins. The presence of
acardiac twin occurs in one of every 35,000 twin pregnancies and in 1% of
all monochorionic twin pregnancies. (1) Other names given are
holocardius, hemicardius, and fetus amorphous, such cases have been
reported in literature as early as 1533. Occurrence of acardiac twin is due
to twin to twin reversed perfusion sequence (TRAP) occurring in
embryogenesis.
II. Introduction
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9. 
There is vascular communications between the twins in monozygotic
twins. The vascular communication of the acardiac twin is different in that,
the acardiac twin receives its blood supply from the other twin pump through
the umbilical artery. The blood in the umbilical artery is mostly
deoxygenated. Hence it leads to a secondary organ atrophy.
II. Introduction
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10. 
Upper body does not develop at all hence missing heart and head.
All the blood supply to the cardiac twin derived from the pump twin. The
acardiac twin develops only the lower part of the body or just a mass of
tissue. Hence the mortality for the acardiac twin is 100%.
Thus a case of 26 year old women with acardiac twin pregnancy
diagnosed by ultrasound is presented.
II. Introduction
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12. 
 Monozygotic multiple gestations with acardiac twin is uncommon thus
knowledge of this condition has grown slowly. Thus this case aims to
achieve the following:
1. To present a case of monozygotic multiple gestation with acardiac twin
2. To determine the sign, symptoms and risk factors of acardiac twin
pregnancy
3. To determine the proper management and treatment of acardiac twin
pregnancy
III. Objectives
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14. 
 NAME: Patient RBML
 ADDRESS: Sitio Camanggaan, Bersamin, Alcala Pangasinan
 BIRTHDATE: January 12, 1990
 AGE: 26 year old
 GENDER: Female
 NATIONALITY: Filipino
 RELIGION: Roman Catholic
 ADMISSION DATE: 11/29/2016 (6:45 PM)
 SOURCE & RELIABILITY: The primary source of data is the patient herself
with a reliability of 90%
IDENTIFYING DATA
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15. 
Labor Pain
CHIEF COMPLAINT
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16. 
2 days PTA:
The patient went to a lying in clinic for a consultation
because of frequent uterine contractions and was
advised to undergo abdominal ultrasound but was not
done.
HISTORY OF PRESENT ILLNESS
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17. 
1 day PTA:
The patient sought consult at villaflor medical group
satellite clinic at Urdaneta city for her regular check-
up and was discovered that one of her twins was
acardiac.
HISTORY OF PRESENT ILLNESS
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18. 
4 hrs. PTA:
The patient experienced onset of on and off
hypogastric pain accompanied by scanty vaginal
bleeding, sought consult at R1MC, hence
admitted.
HISTORY OF PRESENT ILLNESS
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19. 
Patient had a total of 7 prenatal visits
Ferrous sulfate
Patient does not smoke nor drink alcoholic beverages
PRENATAL HISTORY
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20. 
OB HISTORY
M – 11 years
old
I – 28-30 days
(regular)
D – 4 days
A – 3
pads/day
S – no
dysmenorrhea
Family
Planning –
OCP intake
Number of
partners – 1
Coitarche – 19
years old
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21. OB Score – G2P1 (1001)
OB HISTORY
LMP – April 10, 2016
AOG – 33 3/7 weeks
EDC – January 10,2017
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22. 
Date AOG Manner Place Gender Weight Status Complications
G1 2012 Term NSD Bayambang
District
Hosp.
Female AGA Well
baby
none
G2 Present pregnancy
OB HISTORY
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23. 
 Patient had no history of any admission to any health institution or
having any morbid disease
PAST MEDICAL HISTORY
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24. 
No history of Diabetes, asthma, heart disease,
Tuberculosis, cancer or any other congenital defect
and genetic diseases as claimed by the patient
FAMILY HISTORY
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25. 
 General: (-) fever, (-) weight loss, (-) body weakness, (-) loss of appetite,
(-) easy fatigability
 Skin/nails/hair: no rash, dryness, pruritus, hair loss
 Head: No previous injuries noted
 Eyes: (-) pain, double vision, (-) use of glasses/lens, (-) hyper-
lacrimation, (-) redness, (-) blurred vision
 Ears: no abnormal discharges noted
 Nose & sinuses: no abnormal discharges noted
REVIEW OF SYSTEMS
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26. 
 Throat: : (-) use of dentures, (-) bleeding gums, (-) hoarseness, (-) toothache,
(-) mouth sores, (-) sore throat, (-) dysphagia
 Neck: (-) pain, (-) lump, (-) stiffness
 Respiratory: (-) cough, (-) hemoptysis, (-) dyspnea
 Cardiovascular: no palpitations
 Gastrointestinal: (-) loss of appetite, (-) vomiting, (+) hypogastric pain, (-)
hematochezia, (-) hemorrhoids, (-) nausea, (-) hematemesis, (-) dysphagia, (-
) diarrhea, (-) constipation
 Renal: (-) dysuria, (-) nocturia, (-) incontinence, (-) polyuria, (-) gross
hematuria, (-) urinary retention, (-) tea colored urine
 Genitalia: (-) pain, (+) discharge, (+) vaginal bleeding, (-) mass, (-) ulcers
REVIEW OF SYSTEMS
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27. 06/05/2020 27

28. 
 Patient is well groomed, endomorphic, in lying position, who is awake,
alert and responds quickly and appropriately to questions. The patient
is oriented to time, place and person.
 Vital signs:
PR: 89 bpm
RR: 20 cpm
TEMP: 36.3 C (axillary)
BP: 120/80 mmHg
General Appearance:
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29. 
Examination of the Skin:
SKIN: No clubbing of nails. No rash, petechiae or
ecchymosis. Warm, without jaundice, no rashes. With
good capillary refill of 1-2 seconds.
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30. 
Examination of the HEEADS:
 HEAD: Hair of average texture. Scalp without lesions.
 EYES: Conjunctiva pink; sclera white. Pupils are round, regular, equally
reactive to light. Extraocular movements intact
 EARS: Hearing intact. Left and Right canal are both clear.
 NOSE: pink, septum midline. No sinus tenderness
 MOUTH: Oral mucosa pink. Tongue midline. Pharynx without exudates.
 NECK: trachea midline, no cervical nodule enlargement
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31. 
 Examination of the Respiratory and Cardiovascular system:
 Chest expansion is symmetrical, not in respiratory distress and no use of
accessory muscles, no adventitious sound noted.
 Broncho vesicular sounds over the lung fields.
 Regular cardiac rhythm, normal rate noted.
 Normal S1- S2 noted. No heave, no thrills, and no murmurs.
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32. 
Examination of the Abdomen:
Globular, firm, no scars, with hypoactive bowel
sounds, non-tender.
Fundic Height of 28 cm
Fetal Heart tone of 140 bpm
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33. 
Examination of the Genitourinary tract:
grossly female, no abnormalities noted on inspection of the vagina. Vaginal
examination was unremarkable, with no tenderness or masses noted.
Internal Examination
External genitalia is grossly female in appearance, cervix is 3cm dilated in
beginning effacement, adnexa is free, vagina is grossly normal, with Show,
Intact membranes, with adequate amniotic fluid, presenting part is
cephalic, position is vertex and station -3.
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34. 
 Examination of the Musculoskeletal Tract:
No discharges or foul smells noted. Normal capillary filling
in nail beds, no, no masses and lumps, good muscle tone
and strength. With some congenital malformations noted on
the patient’s several digits.
 Leopold’s Manuever:
LM1: soft binodular mass (buttocks)
LM2: Hard smooth and curved at the left side and nodular
masses at the right
LM3: movable (unengaged)
LM4: flexed
Fetal Lie: longitudinal
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35. 06/05/2020 35

36. 
 Twin reversed arterial perfusion sequence is one of rare occurrence.
 The pump twin pumps blood in reversed way, through umbilical
artery, to the acardiac twin.
 The acardiac twin suffers 100% mortality.
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37. Acardiac twin is classified according to the degree of cephalic and truncal maldevelopment
 Hemiacardius – if the heart is incompletely formed
 Holoacardius – if the heart is absent
I. ACARDIUS-ACEPHALUS
-It is most common variety.
-no cephalic structures are present.
-Head and upper extremities are lacking
-This is the type seen in the present case.
II. ACARDIUS-ANCEPS
-It is highly developed form.
- some cranial structure and neural tissue or brain tissue is present.
-The body and extremities are also developed.
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38. 
III. ACARDIUS-ACORMUS with cephalic structure, but no truncal
structures are present.
-The umbilical cord is attached to the head.
-It is rarest form of the acardia
IV. ACARDIUS AMORPHOUS with no distinguishable cephalic or
truncal structure.
-It is least developed and not recognizable as a human form with
minimal development
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39.  In monochorionic pregnancies,
anastomotic vessels are established
connecting the two circulations.
 Retrograde perfusion via the
anastomotic channel prevents the
normal cardiac development due to
lack of sufficient oxygenated blood.
 The cardia, if develops, is either
tubular or completely infantile.
Thus, the acardiac fetus becomes
dependent on the perfusion of the
“pump” twin.
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40. 
DIAGNOSIS OF ACARDIAC TWIN
Ultrasound Doppler
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41. 
• Deep placental anastomoses in early
embryogenesis cause malformation of
the acardiac twin
• Primary defect in embryogenesis in
one twin leads to failure of cardiac
development.
Acrania Acardia
PATHOGENESIS
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42. 
 A study done Moore et al. concluded that preterm delivery was strongly
associated with the development of hydramnios and congestive heart failure in
the pump twin.
 If the twin- weight ratio was above 70%, the incidence of preterm delivery was
90%; hydramnios was 40%; and pump-twin congestive heart failure was 30%.
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43. 
This suggests that estimation of the relative weights in acardiac twins
provides prognostic information regarding the outcome.
 Poor outcome occurs with congestive heart failure and hydramnios in
the normal twin conservative treatment is done when acardiac twin is
small in size.
 Invasive treatment is required when pump twin is having cardiac
failure to improve the perinatal outcome.
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44. 
Many methods of management have been proposed including
1. Termination of pregnancy
2. Serial ultrasound scans to monitor for signs of decompensation,
medical management of polyhydramnios
3. Serial amniocentesis
4. Hysterotomy to remove anomalous twin
5. Invasive procedures
Goal of prenatal treatment is to stop blood flow to the acardiac twin
without affecting the pump twin in order to improve its outcome.
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45. 
 Platt, et al. in 1983 were the first to suggest occlusion of the circulation to the
acardiac twin as the definitive treatment to interrupt blood supply to it.
Minimally, invasive intervention methods are through cord occlusion
techniques or intrafetal ablation.
 Fetoscopic Cord occlusion has been attempted by embolization, cord ligation,
laser coagulation, bipolar diathermy and monopolar diathermy
 Intrafetal ablation is performed with alcohol, monopolar diathermy,
interstitial laser and radiofrequency.
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46. 
Radio-frequency ablation
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47. 
 Sepulveda W, et al. advocated expectant management in all cases.
 They reported 90% survival in pump twin in 10 pregnancies with an acardiac
twin managed expectantly. They cautioned against aggressive intervention and
recommended expectant management with close fetal surveillance.
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48. 
 Sepulveda W, et al. gave an opinion that conservative management is
indicated in cases where the acardiac twin is small and when there are no
signs of cardiovascular impairment in the pump twin.
 Serial ultrasound surveillance is important for detecting any worsening of
the condition, which may suggest the need for interventions to optimize the
pump- twin’s chance for survival.
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49. 06/05/2020 49

50. 
Acardiac twin is a rare case, severe congenital malformation seen in
monozygotic twin gestation which is incompatible with life. Early detection
of acardiac twin can be done in the 1st trimester. Ultrasound and Doppler are
essential in initiating timely treatment and preventing complications such as
cardiac failure in pump twin, preterm labor; thereby improving the perinatal
outcome. First line of treatment is by blocking the vessel of the acardiac twin
by radio frequency ablation by ultrasound guidance. The interruption of
vascular communication between the twins is difficult to accomplish.
VI. SUMMARY & CONCLUSION
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51. 
It needs expensive equipment and trained personnel for the
procedures. Such pregnancies should be managed by the fetal medicine
personel better than cord occlusion techniques. Intra fetal ablation
procedures are better than cord occlusion techniques. In my case, the
pump twin didn’t survive due to lack of fetal surveillance and invasive
treatment in improving perinatal outcome.
VI. SUMMARY & CONCLUSION
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52. 
Thank you for Listening!
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