Volume 1 Issue 7, July 2014

Editorial
Parulekar SV

Torsion of huge benign serous cystadenoma
Bakre T, Gupta AS.

Abdomino-Uterine Adhesion
Niphadkar M, Parulekar SV, Mirchandani A.

Periabortal Abdominal Tuberculosis
Patel A, Qureshi S, Gupta AS.

Uncorrected d-transposition of great arteries in pregnancy
Poonia S, More V, Satia MN.

Successful Management of Ovarian Hyperstimulation Syndrome
Khadkikar R, Pawde A, Saxena N, Chauhan AR.

Forgotten Intrauterine Contraceptive Device as an Uncommon Cause of Postmenopausal Bleeding
Chakre S, Mayadeo NM, Pardeshi S.

Angiomyofibroblastoma of the Vulva
Patil A, Parulekar SV, Samant PY, Fernandes G.

Spontaneous Hemoperitoneum Secondary To Warfarin Toxicity
Saxena N, Chauhan AR.

Editorial

Parulekar SV

This issue of the Journal of Postgraduate Gynecology  Obstetrics is a fine mix of articles from different areas of the subject. Usually very large ovarian tumors do not undergo torsion, because there is just not enough space left in the abdominal cavity for a torsion to take place. Here is a case in which a tumor weighing 3.75 kg underwent torsion. Another area of concern in Gynecological surgery is development of postoperative adhesions. These adhesions are more intense after operations that produce scars on peritoneal surface of the uterus, as in myomectomy. Adhesions of the uterus with the back of the anterior abdominal wall are not common even after myomectomy, because the uterus is nearly normal-sized at the end of the surgery and does not come in contact with the abdominal wall during the process of healing. They are less common after cesarean sections, because the uterine scar is extraperitoneal, and the large puerperal uterus with an intact serosa does not permit development of such adhesions. But we have seen a few cases where the uterus was totally adherent to the anterior abdominal wall, and a repeat cesarean section in those cases was virtually extraperitoneal. Here we present a case of such adhesions encountered during an abdominal hysterectomy and a novel way of handling such adhesions. Tuberculosis continues to be a menace in some Asian countries, especially the multi-drug resistant type. It also continues to be an important cause of maternal mortality. We present a case which was managed very well and survived the serious disease. Congenital heart disease should be treated in childhood, and no girl child should reach adulthood and get pregnant with an uncorrected heart disease. Unfortunately healthcare is too expensive or inaccessible in some parts of the country and such cases do turn up. Here we present a case whose management was quite taxing, but the results were equally rewarding. In the area of assisted reproduction, ovarian hyperstimulation is the norm. Unfortunately, sometimes it is done by persons not adequately trained to do so, and the patient develops ovarian hyperstimulation syndrome. Here is a case of that type, which was successfully managed. Postmenopausal bleeding from the uterus can have varied etiology. Forgotten intrauterine device can be one of them, though the question that remains unanswered is how a woman could forget the intrauterine device she had got inserted. Here is a case in which the diagnosis was missed even on ultrasonography, and was made during a fractional curettage. Vulvar angiomyofibroblastoma is a rare but interesting tumor. The diagnosis is critical, because unlike others that it can be mistaken for, it has a very good prognosis after its removal. Such a case is presented here. The previous issues of the journal have carried articles on hemoperitoneum in women on anticoagulation. A case of spontaneous hemoperitoneum in such a woman is presented in this issue. I hope articles on such varied aspects of Gynecology and Obstetrics will interest our readers.

Torsion of Huge Benign Serous Cystadenoma

Author Information

Tejashree Bakre*, Gupta AS**
(*Third Year Resident, ** Professor. Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

A case of  a huge serous cystadenoma incidentally detected during pregnancy followed by a full term normal vaginal delivery and undergoing torsion 7 weeks  after parturition is presented. Exploratory laparotomy revealed hemorrhagic necrosis due to torsion of the huge serous right ovarian cyst.

Introduction

Adnexal masses occur in 1 out of 200 pregnancies.  Commonest benign adnexal mass is a cystic teratoma followed by a cystadenoma.[1] Serous cystadenomas can grow enormously and fill the entire abdominal cavity as seen in the present case.  Torsion and rupture of the cyst are the common and dreaded complications. Torsion usually occurs either in the late first trimester or second trimester of pregnancy or in puerperium as the ovarian tumor becomes an abdominal organ and has space to freely move and tip over.  Rupture of the cyst can be spontaneous or following increased intracystic pressure due to torsion. Here we present a case of a huge serous cystadenoma incidentally detected during pregnancy followed by a full term normal vaginal delivery and undergoing torsion 7 weeks  after parturition.

Case Report

A 24 year old married woman who had delivered 50 days ago, presented with complaints of abdominal distension, severe stabbing pain in right iliac fossa for 4 hours radiating to back and accompanied with one episode of vomiting. Ultrasonography (USG) done in her 6th month of pregnancy had diagnosed a large benign cystic lesion of the right ovary. The cyst had gradually increased in size but was asymptomatic and the patient had an uncomplicated  normal delivery in a private hospital. From delivery to presentation in the casualty patient experienced several episodes of mild to moderate dull aching pain in the lower abdomen. On presentation, general condition was fair. The patient’s temperature was normal, pulse was regular, 90 beats per minute, blood pressure was 110/70 mm of Hg, and there was no pallor, icterus or edema. Systemic examination was normal. On abdominal examination a 34 weeks’ size, cystic, tender, mobile mass arising from pelvis was felt. There was no guarding or rigidity. On bimanual pelvic examination the lower end of the cyst could not be felt, the uterus was anteverted and normal sized, right fornix was tender and left fornix was clear but non tender An USG was done. It showed a pelvic anechoic mass extending towards the upper abdomen of size 20.5 x 16.4 x 14.8 cm with internal echoes, debris and septations in the right adnexa arising from the right ovary and extending up to the right hypochondrium; most likely a right ovarian mucinous cystadenoma. The left ovary and uterus were normal. Doppler study revealed a right ovarian cyst with peripheral compressed follicles with twisting of the vascular pedicle and presence of Whirlpool sign. A computerized tomography (CT) confirmed the diagnosis, but did not reveal any evidence of torsion. Reports of tumor marker tests were CEA: 1.38 ng/ml, CA 125: 33.2 U/ml and CA 19: 5.78 mcg/ml. All were within normal range. Torsion of the ovarian mass or hemorrhage in the ovarian mass was provisionally diagnosed. Her hemoglobin was 11.7 gm%, white blood cell count was 14,300/mm3, blood urea nitrogen, serum creatinine and serum electrolytes were normal. An exploratory laparotomy was done under general anesthesia. Abdomen was opened through an infraumblical incision. No ascites was seen. Peritoneal fluid saline washings were sent for cytology. A large dark, cyanosed, blackish brown, hemorrhagic ovarian cyst was seen filling the entire peritoneal cavity. Incision was extended cephalad to deliver the cyst. A 23 x 20 x 20 cm cyst with smooth surface arising from the right ovary and having two and half circle clockwise torsion of pedicle was noted as seen in Figure 1. The pedicle was untwisted but the color did not improve. A right salpingo-oophorectomy was done. The uterus, left ovary and fallopian tube were normal. Liver, spleen and bowels were normal. The cyst weighed 3.75 kg. On cut section, the cyst did not show any solid component or papillary excrescences. It was bilocular with septations and thick congested walls. Several rubbery blood clots blackish in color were seen. Approximately 400 ml of brownish black fluid was drained from the cyst. The patient had an uneventful postoperative recovery and was discharged on 5th day. Peritoneal fluid cytology was negative for malignant cells.  Histopathology reported a bilocular cyst with brown gelatinous material, thick congested smooth walls with flattened to cuboidal epithelium resting on fibro-collagenous stroma with areas of recent and old hemorrhages. The final diagnosis was  serous cystadenoma with hemorrhagic necrosis due to torsion.

Figure 1. Torsion of the Ovarian Cyst. C is the necrotic ovarian cyst. Arrow shows the twisted pedicle.

Figure 2. The excised huge cyst.

Discussion

Ovarian torsion is twisting of ovary alone or more often along with its salpinx.  This twisting initially decreases its venous drainage. This results in increased pressure which on exceeding the arterial pressure will cause necrosis and infarction of the ovary by interrupting the arterial flow.[2]
Asymmetrical enlargement of the ovary occurs due to the cyst resulting in a weight imbalance causing the ovary to topple over. This twists its pedicle along the long axis of its salpinx.[2] Ovarian cysts with sizes ranging from 6 to 8 cm have the highest chances of undergoing torsion. However, in our patient a tumor with a diameter of 20 cm rotated on its axis. Such large tumors have space in the abdominal cavity during puerperium due to the lax abdominal wall. Our patient had all the typical presentation of an ovarian torsion. She had sudden onset of severe, sharp stabbing, and unilateral lower abdominal pain worsening intermittently over many hours. She had no nausea and vomiting that occurs in 70% of such patients. She had history of repeated episodes of abdominal pain that can be attributed to partial spontaneously resolving torsion. Temperature elevation is seen as the ovary becomes necrotic.[3] Color Doppler USG is the investigation of choice to evaluate  adnexal torsion. The  physiologic and morphologic changes that result due to impaired blood flow in a  twisted ovary are seen on Doppler.[4,5]  Normal Doppler findings do not exclude an adnexal torsion.  CT and magnetic resonance imaging are additional diagnostic techniques when USG findings are non diagnostic. Torsion of an ovarian cyst is a surgical emergency warranting an operative laparoscopy or laparotomy. Laparoscopic gynecologic surgery is safe in first trimester of pregnancy.[6] Laparoscopy  has also been used for removal of very large serous cystadenomas in advanced pregnancy[7]. Our patient should have undergone an exploratory laparotomy in the second trimester of pregnancy when the cyst was diagnosed or immediately after delivery in the puerperal period. The delay resulted in growth of the cyst, torsion, ischemic infarction and loss of an endocrine organ  in a young woman in the early 3rd decade of her life. This line of management is well documented in literature.[8]

Conclusion

With increasing use of USG,  ovarian cysts with pregnancy are easily detected. All ovarian cysts during pregnancy should be followed by USG.  If cysts fail to regress or they grow, surgical intervention is required. This can certainly prevent complications like torsion after delivery when its incidence is high due to involution of the uterus. Moreover presentation of ovarian torsion can be misleading. Even investigations like color Doppler and CT scan have diagnostic fallacies. High vigilance, early detection and prompt action can avoid tragic consequences.

References

1. Mandi D, Mondal RC, Bhar D, Maity AK, Nandi MK, Sing K. Successful vaginal delivery despite a huge Ovarian Mucinous cystadenoma complicating pregnancy: A Case Report. Iran J Med Sci. Dec 2013;38(4):339–342.
2. Murphy T. Ovarian torsion. Available from www.columbiabasinherald.com/ community/ healthtips/ article_87457b0b-a3bc-5456-850f-cd4e916d0c5e.html
3. Vellanki V S, Sunkavalli CB. Giant ovarian serous cystadenoma in a postmenopausal woman: a case report. Cases J. 2009;2:7875.
4. Kolluru V, Gurumurthy R,  Vellanki V and Deshpande G. Torsion of ovarian cyst during pregnancy: a case report. Cases Journal 2009,2:9405.
5. Lee EJ, Kwon HC, Joo HJ, Suh JH. Diagnosis of ovarian torsion with color Doppler sonography. J Ultrasound Med Feb 1998;17(2):83-9.
6. Schraga ED, Fleischer AC. Ovarian Torsion. emedicine.medscape.com/ article/ 2026938.
7. Johnson JR, Lee C, Carnett S, Vadakekut E. Laparoscopic management of enlarged serous cystadenoma in advanced pregnancy.  J Minim Invasive Gynecol 2007;14(2):247-9.
8. K Shah, S Anjurani, V Ramkumar, P Bhat, M Urala. Ovarian Mass In Pregnancy: A Review Of Six Cases Treated With Surgery. The Internet Journal of Gynecology and Obstetrics. 2009 Volume 14 Number 2. Available from http://ispub.com/IJGO /14/2 / 12324 .

Citation

Bakre T, Gupta AS. Torsion of huge benign serous cystadenoma. JPGO 2014 Volume 1 Number 7 Available from: http://www.jpgo.org/2014/07/torsion-of-huge-benign-serous.html

Abdomino-Uterine Adhesion

Author information

Manamita Niphadkar*, Parulekar SV**, Mirchandani A***

(*Second year resident, **Professor and head of the department, *** Assistant Professor Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Pelvic and intra abdominal adhesions are known post-operative complications in patients who have undergone cesarean section or any other abdominal surgery.^[1,2] We report a case of an unusual adhesion of the uterus to the anterior abdominal wall in a 44 year old woman with history of previous 2 cesarean deliveries undergoing total abdominal hysterectomy.

Introduction

Intraperitoneal adhesions is a known complication of any abdominal surgery.^[1,2] Most of the adhesions are between the uterus and omentum.^[3] Sometimes bowel may get adherent to the uterus.^[1,2,4] Development of adhesions between the uterus and the anterior abdominal wall in not very common. However this complication is on the rise with increase in the rate of multiple cesarean sections.^[4] Such adhesion may lead to difficulty in future abdominopelvic operations by limiting access. We report a case of an unusual adhesion of the uterus to the anterior abdominal wall.

Case report

A 44 year old woman, married for 30 years, with previous 2 cesarean sections, presented with complaints of menorrhagia for 3-4 years. Her menses occurred every 28-30 days, lasting for 6-8 days, soaking 4 pads/day, associated with passage of clots and dysmenorrhea. She had undergone a hysteroscopy with dilatation and curettage for the same 10 months ago. Endometrial histopathology report showed proliferative changes. She had mild pelvic inflammatory disease clinically, and was cured of it with a course of doxycycline and metronidazole. She was a known case of ulcerative colitis on treatment with sulfasalazine. Colonoscopy was done in 2008 which showed grade 3 changes up to 25 cm from anal verge.

There was no history of any other major medical or surgical illness. Her uterus was mildly enlarged, high in position and fixed. Traction on the cervix vaginally produced retraction of the anterior abdominal wall midway between the umbilicus and pubis. There was a Pfannensteil scar. General and systemic examination revealed no abnormality. Her hemogram, blood sugar, liver function tests, renal function tests, chest radiograph and electrocardiogram were normal.

An abdominal hysterectomy was scheduled. The abdomen was opened in layers by infraumbilical vertical midline incision till the rectus sheath. Rectus sheath was found to be firmly adherent to the anterior wall of the uterus and the uterus was pulled up due to adhesions. There were omental adhesions to the uterus, which were dissected free. The left round ligament was identified, while the right one could not be seen. The left round ligament was divided and ligated with No. 1 polyglactin transfixion suture. Then the  lower limit of the adhesion to the anterior uterine wall could be reached, and a finger could be passed between the uterovesical pouch of peritoneum and the lower limit of the adhesion, which was below the level of the isthmus. The uterus was dissected sharply from the abdominal wall from the left to the right, and the freed portion was swung backwards as the separation progressed. Finally the left round ligament could be clamped, cut, and ligated. The subsequent steps of hysterectomy were as in the conventional technique.

Figure 1. The uterus is adherent to the anterior abdominal wall. A. Inability to insert the C-shaped retractor between the uterus and the anterior abdominal wall on the right side indicates total occlusion of that area; B. Finger in the cavity of the rectus sheath.

Figure 2. Omental adhesion with the uterus elevated with an index finger (shown by the black arrow).

Figure 3. Application of straight clamps to the cornual structures.

Figure 4. Omentum dissection from the right side of the uterus.

Figure 5. Separation of the bowel adherent to the right side of the uterus.

Figure 6. Division of the left round ligament. The part of the anterior peritoneal pouch between the utero-vesical fold of peritoneum and lower limit of the adhesion is shown by the white arrow.

Figure 7. Separation of the right side of the abdomino-uterine adhesion after swinging the left part of the uterus backwards.

Figure 8. The adhesion has been dissected. The right round ligament is shown by a white arrow.

Figure 9. The right round ligament has been divided (black hollow arrows). The raw area on the anterior uterine surface is shown by white arrows. The free part of the anterior peritoneal pouch lies at the level of the solid black arrow.

Figure 10. The anterior surface of the specimen after hysterectomy.

Discussion

The incidence of adhesions between the posterior surface of the anterior abdominal wall and the anterior surface of the uterus has been rising owing to the increased rates of caesarean sections in the last few decades.^[1,2] It is further increased proportional to the number of cesarean sections a woman undergoes.^[4] If the area of the adhesion is long and wide, the lower part of the anterior surface of the uterus cannot be approached. Since the location the urinary bladder in these adhesions cannot be predicted, it is at risk of injury during separation of the adhesions. We dissected the adhesion on one side, divided the round ligament on that side, and reached the lower lateral limit of the adhesion. After ascertaining the location of the bladder, dissection was continued to the other side. This technique is safer than starting the dissection at the upper part of the adhesion and progressing downwards towards the bladder.

From the study of a single case, one cannot conclude about closure or non closure of the uterovesical fold of the peritoneum and its effect on such adhesions. The operation notes of the cesarean section of this patient were not available to help decide the cause of the adhesion. It was not an inverted T-shaped incision on the lower segment, as the part above the uterovesical fold was free. It is possible that the anterior surface of the uterus was injured while a deep incision was made on the anterior abdominal wall. The vertical suture line on the uterus and the closed abdominal wall incision might have resulted in this adhesion, as would occur in the case of an upper segment cesarean section.

References

1.      El-Shawarby S, Salim R, Lavery S, Saridogan E. Uterine adherence to anterior abdominal wall after caesarean section. BJOG 2011;118:1133–1135.

2.      Stark M, Hoyme UB, Stubert B, Kieback D, di Renzo GC. Post-cesarean adhesions - are they a unique entity? J Matern Fetal Neonatal Med 2008;21:513–6.

3.      Awonuga AO, Fletcher NM, Saed GM, Diamond MP. Postoperative Adhesion Development Following Cesarean and Open Intra-Abdominal Gynecological Operations. Reproductive Sci. 2011 December; 18(12): 1166–1185.

4.      Z Shi, L Ma, Y Yang, H Wang, A Schreiber, X Li, et al.  Adhesion formation after previous caesarean section-a  meta-analysis and systematic review. BJOG 2011;118:410–22.

Citation

Niphadkar M, Parulekar SV, Mirchandani A. Abdomino-Uterine Adhesion. JPGO 2014 Volume 1 Number 7 Available from: http://www.jpgo.org/2014/07/abdomino-uterine-adhesion.html

Periabortal Abdominal Tuberculosis

Author Information

Patel Amit, Qureshi Shabnam, Gupta AS.

(*Second Year Resident, ** Assistant Professor, *** Professor. Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Pregnancy is an immunosuppressive state in which the immunity of the patient, especially the cell mediated immunity is suppressed to allow implantation and development of the zygote.

With termination of pregnancy, there is a flare up of quiescent infection so that it becomes

symptomatic. We present a case of a postabortal patient who presented with fever and abdominal pain after check curettage done for incomplete abortion. She was suspected to have postabortal sepsis, flare up of pelvic inflammatory disease or genital tuberculosis. CT scan report clinched the diagnosis of abdominal tuberculosis.

Introduction

15-20% of extra pulmonary tuberculosis is genital tuberculosis.^[1] Though not very common genital tuberculosis can initially present after obstetric procedures like check curettage for

in complete abortion or in the puerperium. The patient mostly comes with fever and pain in abdomen. Differentiating between postabortal/puerperal sepsis, flare up of pelvic inflammatory disease or genital/abdominal tuberculosis is challenging.

Case report

A 24 year old patient gravida 3, para 1 and 2 abortions presented with complaints of abdominal pain and progressive abdominal distention. It was precipitated after a curettage was performed in a private set up for incomplete first trimester spontaneous abortion. Patient developed low grade fever and abdominal pain on the second post operative day. There was no history of vaginal discharge or burning micturition. Patient consulted multiple private consultants prior to coming to us. She underwent multiple investigations and received broad spectrum antibiotics but had no clinical improvement. On admission she had abdominal pain and vomiting. She was afebrile. She had no foul smelling vaginal discharge, urinary complaints. There was no history of cough or previous tuberculous infection in her or in her family. General examination parameters were normal. Her abdomen was mildly distended and it felt doughy on palpation. Mild tenderness was elicited in the right iliac fossa. On speculum examination cervical os was closed. No bleeding was seen. Cervix and vagina appeared healthy. On bimanual vaginal examination the uterus was normal in size, bilateral fornices were free and no cervical motion tenderness could be elicited.

The results of her investigations are tabulated in the table below. On admission with us the patient had the reports that are shown in the 1^st 5 columns of the investigation chart. 

Investigations Chart

	2-4-14	3-4-14	8-4-14	11-4-13	17-4-14	21-4-14
Haemoglobin g%	12.3	12.6	12.7	12	10.3	9.0
Total WBC count/mm3	9700	12000	12600	11800	10200	14000
Platelet count lac/mm3	2.53	Adequate	3.10 lac	3.51	Adequate	4.52
ESR			2 mm at 1hr
HIV	negative
VDRL	negative
HbsAg	negative					negative
Leptospirosis	negative					negative
Dengue	negative					negative
PS for Malarial parasite	negative					negative
Malarial antigen test	negative					negative
Widal	negative
Urine routine	WNL		WNL		WNL	WNL
Urine culture			No growth
Blood culture			No growth
Tb Gold			Negative
Ultrasonography (USG) Abdomen and pelvis  It showed a mild bulky uterus, mild fluid in POD and minimal ascites.
CT scan of the abdomen and the pelvis showed locculated ascites and thickening of the peritoneum, multiple mesenteric lymph nodes with extensive stranding, and omental calcification. These features were suggestive of peritoneal Koch’s with mesenteric adenopathy.

Figure 1: CT scan shows the enlarged multiple mesenteric lymph nodes (N).

Figure 2: CT scan shows the locculated ascites (A) and extensive mesenteric stranding (M)

CT scan was suggestive of abdominal Koch’s. The patient was referred to a gastroenterologist who started her on category-2 AKT. She is following up with the gastroenterologist and her symptoms have reduced. 

Discussion

Pregnancy is an immunosuppressive state. This facilitates embryonic implantation. At the end of pregnancy rapid reversal of this immunosuppressive state occurs. This is due to reactivation of the pre-inflammatory substances. This immunocompetent state allows rapid flaring up of those diseases that are dependent on the immune system. Tuberculosis is one of them. The main pathology in tuberculosis is cell mediated immunity against the tubercular antigen, that is suppressed during pregnancy. So as soon as pregnancy is completed latent infection activates and produce symptoms. Lymphocyte activity reactivates within 24 hours and recovers completely within 4 weeks. In previous trials it is seen that tuberculosis becomes symptomatic within 10 days in 76% of cases.^[2] In our case activation occurred on day 2 after abortion. The quiescent mycobacterium tuberculosis infection flared up and the patient became symptomatic immediately. As the patient had an early (1^st trimester) pregnancy loss, probably her cell mediated immunity suppression reverted back faster.

Conclusion

This case is presented, as tuberculosis is a very important ailment. The clinician has to be alert to its flare up in the immediate peripartum state as delay in diagnosis increases patient morbidity and unnecessary use of empirical antibiotics.

References

1.      Rajamaheshwari N . Pelvic tuberculosis. Available from: http://www.sunmed.org/pelvictb.html

2.      Cheng VC, Woo PC, Lau SK, Cheung CH, Yung RW, Yam LY, et al. Peripartum tuberculosis as a form of immunorestitution disease. Eur J Clin Microbiol Infect Dis 2003;22(5):313-7.

Citation

Patel A, Qureshi S, Gupta AS. Periabortal Abdominal Tuberculosis. JPGO 2014 Volume 1 Number 7 Available from: http://www.jpgo.org/2014/07/periabortal-abdominal-tuberculosis.html

Uncorrected d-Transposition of Great Arteries in Pregnancy

Author Information

Supriya Poonia*, Vibha More**, M. N. Satia***

(*Third Year Resident, ** Assistant Professor, *** Professor. Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)

Abstract

Congenital heart diseases (CHD) are the most common birth defects. They affect approximately 0.8% of all live births.[1] They are an important cause of maternal and perinatal morbidity and mortality. We present an important case of 22 years primigravida with previously diagnosed but uncorrected case of d-Transposition of Great arteries (TGA) who delivered full term low birth weight baby at 38 weeks of gestation.

Introduction

TGA accounts for 5-7 % of congenital heart disease.^[2]   TGA is a birth defect in which two main outlet arteries – aorta and pulmonary artery are transposed i.e. switched in position. In babies with TGA deoxygenated blood enters the right side of heart and instead of going to the lungs, is directly pumped into systemic circulation through aorta. Often babies with d-TGA have other congenital heart defects, such as ventricular septal defect (VSD) or atrial septal defect (ASD) or patent ductus arteriosus (PDA) which are necessary for mixing of blood between two parallel circulations and for survival. Because a baby with TGA cannot maintain oxygen saturation and requires surgery immediately after birth it is considered as a critical congenital heart disease.

Physiological changes of pregnancy may mimic or mask heart disease. Echocardiography (ECHO) is the first investigation of choice. Many women of TGA are reaching reproductive age group owing to improved survival because of surgical treatment in childhood or may be for the first time picked up later in life due to compensation by other associated lesions.^[3]   Preconception counselling and skilful management with multidisciplinary approach of obstetrician, cardiologist, anaesthetist and neonatologist plays an important role in successful pregnancy outcome.^[4]

Case Report

A 22-year-old primigravida, married for 2 years, was referred at 19.5 weeks of gestation with complaints of dyspnea on excretion NYHA class II. There was a history of easy fatigability since childhood and delayed milestones. Cardiac evaluation was done 1 year ago and the patient was diagnosed to have d-TGA, started on metoprolol 25 mg and advised surgery. She was not compliant to treatment. No further treatment details were available. In present pregnancy she was hospitalised for further evaluation. On admission general condition was moderate, vital parameter were normal, clubbing and peripheral cyanosis were present; oxygen saturation was between 80-85%. On cardiovascular examination showed left parasternal heave, loud second heart sound, pansystolic murmur, and ejection systolic murmur at aortic and pulmonary area.  Respiratory and CNS examination was normal. On obstetric examination the uterus was corresponding to 20 weeks of gestation.  Cardiology opinion was taken. 2D ECHO was done, which showed cyanotic heart disease, situs solitus with double outlet right ventricle d- TGA, ostium secundum atrial septal defect (ASD) with bidirectional shunt, subaortic ventricular septal defect (VSD) with bidirectional shunt, severe infundibular and valvular pulmonary stenosis, and large patent ductus arteriosus (PDA).  Electrocardiograph showed normal sinus rhythm, right axis deviation, tall P wave with right atrial enlargement. She was advised to continue metoprolol and was also started on tablet propanolol 20 mg q8h. Obstetric ultrasonography, fetal anomaly scan and fetal 2D-ECHO were normal. She had secondary polycythaemia with haemoglobin 18 g/dL, packed cell volume (PCV) 53. Hematologist advised to maintain her PCV below 55.  She was advised regular follow-up at antenatal, hamatology and cardiology outpatient departments. At 29 and 32 weeks of gestation the patient had complaints of headache, dizziness, and myalgia. The PCV was 65 and 68. After haematology opinion isovolumic phlebotomy was done. At 34 weeks of gestation she was advised admission in view of intrauterine growth restriction but she did not get admitted. At 38.5 weeks she went into spontaneous labour and was transferred to cardiac intensive care unit. Infective endocarditis prophylaxis was given. Epidural analgesia with fentanyl 25 mcg and 0.1% bupivacaine 6 ml was given. She progressed uneventfully and forceps application was done to cut short second stage of labour. She delivered a female baby weighing 1.8 kg with Apgar score of 9/10. Intravenous diuretics were specifically avoided postdelivey to maintain preload to systemic ventricle. She was monitored in cardiac intensive care unit and discharged on seventh day after delivery. She was counselled to avoid further pregnancy and advised to follow up 6 weeks after delivery in cardiology OPD for Rastelli Repair. Her husband was counselled for vasectomy.

Discussion

The incidence of cardiac disease is less than 1 % in pregnancy. TGA is a one of the critical congenital cyanotic heart disease. TGA can be of 2 types: d – TGA, also called as complete TGA, is the most common type. In this the aorta arises from the morphological right ventricle (RV), and the pulmonary artery arises from the morphological left ventricle (LV), the “d-” refers to the dextro position of the bulboventricular loop. The other type is l-TGA, also called as congenitally corrected TGA, in which the right atrium enters the left ventricle, which gives rise to the pulmonary artery, and the left atrium communicates with the right ventricle, which gives rise to the aorta. Patients who have some route for oxygenated blood to enter from left to right side of heart like VSD and pulmonary vascular disease may reach adulthood without corrective surgery due to Eisenmenger physiology. In cyanotic heart disease, chronic hypoxia causes increased erythropoiesis which leads to increased red cell mass (Polycythaemia) and in turn increased oxygen carrying capacity of blood and heamatocrit (viscosity). Increased viscosity negates oxygen carrying capacity. Phlebotomy is to be done only if symptoms of hyperviscosity such as headache, dizziness, fatigue, chest pain, myalgia, paraesthesiae, depression are present.^[4] TGA is associated with common cardiovascular complication during pregnancy such as arrhythmia, deterioration of NYHA class, thromboembolism, endocarditis, heart failure and obstetrical complications such as preeclampsia and eclampsia, premature rupture of membranes, preterm labour, prolonged second stage of labor, postpartum haemorrhage and small for gestation age infants ,fetal and  perinatal mortality.^[5]   Risk of CHD recurrence in infant is <1%.^[3] .Low oxygen saturation <85% is associated with low live births< 12%.

It is important to conduct proper preconception counselling regarding risk of pregnancy to her health, risk of inheriting the defect and skilful management throughout pregnancy and delivery.^[6] Function of right ventricle and degree of tricuspid regurgitation plays a significant role in predicting the increased volume overload and leads to irreversible right ventricle dilatation.^[7] Periodic follow up with 2D ECHO in third trimester is done to look for worsening in systemic ventricular function; increasing atrioventricular valve or tricuspid regurgitation. In this case d-TGA was picked up in early twenties and was not surgically corrected till delivery. Surgical procedures like atrial (Mustard and Senning procedure) and arterial switch, which are reserved for first few months of life couldn’t be done hence Rastelli procedure was advised. Rastelli procedure is done when d-TGA coexists with a large subaortic VSD and pulmonary stenosis. A patch is placed to direct blood from the left ventricle to the aorta through the VSD. The pulmonary valve is over sewn, and continuity is established between the RV and the pulmonary artery by means of a valved conduit. This is a case where uncorrected d -TGA was successfully managed conservatively in tertiary care centre with multidisciplinary approach and advised surgical correction after delivery.

References

1.      Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890–1900.

2.      Ferencz C, Rubin JD, Loffredo CA, Epidemiology of Congenital Heart Disease: The Baltimore-Washington Infant Study, 1981–1989. Mount Kisco, NY: Futura Publishing Company; 1993.

3.      Warnes CA. Transposition of great arteries. Circulation. 2006;114:2699-2709

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Citation

Poonia S, More V, Satia MN. Uncorrected d-transposition of great arteries in pregnancy. JPGO 2014 Volume 1 Number 7 Available from: http://www.jpgo.org/2014/07/uncorrected-d-transposition-of-great.html