Open Access Case report P regnancy in non-palliated functionally single ventricle: challenges of management in resource-poor settings Desrie Delsol-Gyan1,2,&, Ernest Aniteye2,3, Samuel Oppong3, Ernest Ofosu-Appiah2,3, Frank Edwin1,2 1School of Medicine, University of Health and Allied Sciences, Ho, Ghana, 2National Cardiothoracic Centre, Korle Bu Teaching Hospital, Accra, Ghana, 3School of Medicine and Dentistry, University of Ghana, Accra, Ghana &Corresponding author: Desrie Delsol-Gyan, School of Medicine, University of Health and Allied Sciences, Ho, Ghana Key words: Single ventricle, congenital heart disease, pregnancy Received: 13 Feb 2019 - Accepted: 12 Jun 2019 - Published: 09 Jan 2020 Abstract Women with complex functionally univentricular hearts rarely survive into adulthood without corrective or palliative surgery. Reports of pregnancy outcome in this group of patients in resource-poor settings are sparse. We report a case of unrepaired pulmonary atresia ventricular septal defect (VSD) with major aorto-pulmonary collateral arteries (MAPCA) who survived into adulthood and was able to complete a successful pregnancy in a resource-poor country. Pan African Medical Journal. 2020;35:6. doi:10.11604/pamj.2020.35.6.18442 This article is available online at: http://www.panafrican-med-journal.com/content/article/35/6/full/ © Desrie Delsol-Gyan et al. The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Published by the Pan African Medical Journal – ISSN: 1937- 8688 (www.panafrican-med-journal.com) The Manuscript Hut is a product of the PAMJ Center for Public health Research and Information. Page number not for citation purposes 1 Introduction Patient and observation The term functionally single ventricle refers to a spectrum of Ghana's National Cardiothoracic Centre was officially inaugurated in congenital heart defects in which the ventricular mass, which may be April of 1992. In 1983 when the patient was four years of age, she anatomically single or double, does not lend itself to safe partitioning was part of a selected group of children who received philanthropic in a way that commits one ventricular pump to the systemic support to travel to Europe for treatment of cyanotic CHD. She was circulation, and the other to the pulmonary circulation [1]. Thus a prepared for surgery with a preoperative diagnosis of truncus patient may possess two ventricles and yet manifest single ventricle arteriosus (common arterial trunk). At surgery, a solitary systemic physiology if it is not surgically feasible to partition the circulation in a arterial trunk arising from the ventricular mass was found. The way that commits either ventricle exclusively to the systemic or pulmonary blood flow was derived from large collateral vessels arising pulmonary circulation. Unlike the biventricular circulation in which the from the descending aorta. Because the patient's saturation was systemic and pulmonary circulations are connected in series, in a satisfactory (above 80% most times) no intraoperative intervention single ventricle physiology, the systemic and pulmonary circulations was deemed necessary. The patient returned home after an are connected in parallel, being supplied by the output from the uneventful recovery to be followed up locally. She maintained combined ventricular mass. The ventricular ejection is physiologically satisfactory saturations and fairly adequate exercise tolerance partitioned between the systemic and pulmonary vascular beds throughout childhood. She underwent phlebotomy on three occasions depending on the relative resistances between the two vascular beds. on account of hyperviscosity. Although counselled about the risks of An increase in flow to one vascular bed will only occur at the expense pregnancy, she was insistent on having a child. At age 32 years, she of a reduced flow to the other vascular bed. As a corollary, the oxygen became pregnant but miscarried in the first trimester. One year later, saturation in the aorta and pulmonary arteries are the same. The she presented to our clinic with a seven week pregnancy. specific functionally single ventricle lesions include tricuspid atresia, hypoplastic left heart syndrome, double-inlet left ventricle, pulmonary At the time of the pregnancy, she was not on any medication and had atresia with intact ventricular septum, unbalanced atrioventricular only mild effort intolerance (NYHA II). The oxygen saturations ranged septal defects, and several others. between 86%-89%. Her blood pressure was 101/69mmHg with a pulse rate of 86 per minute. Her hemoglobin was 18.3g/dl (hematocrit Uncorrected pulmonary atresia (PA) with ventricular septal defect of 54.9%). The transthoracic echocardiography was in keeping with (VSD) falls in this category as both the systemic and pulmonary blood her intraoperative findings in childhood: usual veno-atrial and flows are derived the aorta which receives ejection from the combined atrioventricular connections, diminutive outlet portion of the right mass of right and left ventricles through a large sub-aortic VSD. ventricle without continuity with an arterial segment, both ventricles Pulmonary atresia with VSD and major aorto-pulmonary collateral connected to a 40mm solitary arterial trunk through a 27mm sub- arteries (MAPCA) is the most extreme form of tetralogy of Fallot (TOF) arterial VSD. No intra-pericardial pulmonary artery was found. Large representing 5% to 10% of TOF patients [2]. The survival rate without collateral vessels (MAPCAs) arising from the descending aorta surgical repair is reported to be 50% at 1 year of age and 8% at 10 appeared to be the source of pulmonary blood flow, with origin years [3]. Survival into adulthood without palliation is rare; successful stenosis of the left-sided MAPCA. The ventricular function was good pregnancy in such patients is even rarer. Pregnancy is accompanied with ejection fraction of 76%. There were echocardiographic signs of by dramatic changes in systemic vascular resistance and venous pulmonary hypertension. The risks of pregnancy to mother and fetus return from the late second trimester onwards. Pregnancy in patients were once again emphasized and termination discussed as an option with uncorrected PA VSD MAPCA is fraught with many challenges but the patient preferred to continue with the pregnancy. which are magnified in resource-poor settings. These challenges and their management are not usually encountered in routine clinical A multidisciplinary specialist team consisting of a cardiologist, practice in developing countries. Current available guidelines are in obstetrician/gynecologist, and cardiac anesthetist as the core team reference to specialized adult congenital heart disease (ACHD) members was formed to draw a management plan for the pregnancy, centers, largely unavailable in resource-poor settings. We report our deliver, and puerperium. The patient, being a house wife, was advised experience in successfully managing pregnancy in a patient with to reduce her chores at home in keeping with her comfort. The patient uncorrected PA VSD MAPCAs in Accra, Ghana. tolerated the pregnancy relatively well, maintaining oxygen Page number not for citation purposes 2 saturations between 84% and 88% until the 28th week. At 28 weeks diagnosis was truncus arteriosus type IV. The original categorization gestation, her effort tolerance worsened, her oxygen saturation of truncus arteriosus by Collett and Edwards [5] included a type IV dropped to 80% and the hemoglobin rose to 19.9g/dl. She was variant characterized by the complete absence of the intra-pericardial hospitalized and placed on bed rest, close surveillance, and regular pulmonary arteries. Type IV truncus arteriosus is now recognized to fetal monitoring. She was given elastic compression stockings and be a form of PA VSD [6]. anticoagulated with warfarin (target INR between 2 and 3) to counter the risk of thromboembolism. The initial pelvic scan demonstrated no Pregnancy in patients with PA VSD MAPCA carries substantial maternal obvious fetal abnormalities but subsequent scans showed signs of and fetal risks [7] as well as a risk of CHD in the newborn of oligohydramnios. The findings suggested slow amniotic fluid loss. The 13.4% [8]. Surgical intervention in the form of anatomical repair or decision was thus taken at 34 weeks to deliver the fetus. An elective palliation that eliminates or significantly ameliorates cyanosis and Caesarean Section was performed at 34 weeks of gestation under secondary erythrocytosis has a modifying influence on the pattern of general anesthesia. A female infant, weighing 1.2 kg was delivered risk observed in pregnant patients with PA VSD MAPCA. In unrepaired with APGAR scores of 6 and 7 (first and fifth minutes). patients like ours, maternal mortality and morbidity tends to be low while complications occur mainly in the fetus [7]. A high rate of fetal On the second postpartum day the patient developed congestive heart loss is common as occurred with this patient in the first pregnancy. failure. She was treated with diuretics (furosemide and Maternal cyanosis carries substantial risk to the fetus with a higher spironolactone) with good response and was discharged a week later. chance of fetal loss in the first trimester, prematurity, and small-for- The baby was initially nursed in the neonatal intensive care unit. gestational-age neonates. Successful pregnancy is more likely if Echocardiography on the baby prior to discharge showed no CHD. cyanosis is minimal or absent, and erythrocytosis is moderate or less Post-delivery evaluation of the mother showed good ventricular before pregnancy. Both of these are uncommon findings in the function on echocardiogram. A computed tomographic angiography unrepaired patient with PA VSD MAPCA. In one study, the live birth was performed a year after delivery to evaluate the sources of rate was just 8% if the pre-pregnancy hemoglobin exceeded 20g/dl pulmonary blood flow (Figure 1A). The ascending aorta measured whereas it was 71%, if the hemoglobin was 16g/dl or less, if the 45mm with usual branching pattern. The right lung was supplied by maternal oxygen saturation was 85% or less before pregnancy and an 18mm MAPCA from the descending aorta (Figure 1B). The left lung only 12% of infants were born alive [9]. In this regard, our patient was supplied by another MAPCA measuring 20mm with origin stenosis had a borderline fetal risk. (Figure 1C). After six years post-delivery follow up, both mother and child are doing well. The mother's effort tolerance has diminished The systemic and pulmonary blood flows in this patient were derived moderately but she is still able to care for the child and perform her from the solitary arterial trunk with similar oxygen saturations usual daily chores reasonably well. occurring in both the systemic and pulmonary vascular beds. The cardiac ejection by both ventricles into the arterial trunk is partitioned between the systemic and pulmonary vascular beds according to the Discussion relative vascular resistance between the systemic and pulmonary vascular bed. The circulation, in essence, is that of a functionally single ventricle. A decrease in systemic vascular resistance (vasodilatation) Current guidelines recommend that women with CHD should consult will increase systemic blood flow at the expense of pulmonary blood an ACHD specialist before becoming pregnant. Also, the management flow and peripheral oxygen saturation. As pregnancy advances and of pregnant patients with cyanotic CHD should be undertaken in an systemic vascular resistance falls, hypoxemia may worsen because of ACHD center by a multidisciplinary team that includes ACHD specialists increased systemic blood flow at the expense of pulmonary blood and maternal-fetal medicine obstetricians with expertise in caring for flow [4]. We believe this is what happened to the patient when she women with heart disease [4]. In resource-poor settings, these ideals reached the 28th week of gestation. The worsening hypoxemia and are not attainable due to infrastructure and manpower constraints. erythrocytosis decreased her effort tolerance and further Management of such patients in these settings is often accomplished compromised an already precarious intrauterine growth. Both chronic by drawing upon the expertise of practitioners across various cyanosis and pregnancy are prothrombotic states. When the patient departments in a tertiary facility who must work together to achieve was admitted for bed rest and close monitoring, it was felt that her optimal outcomes for the patient. During childhood, this patient's thromboembolic risk was heightened and so compression stockings Page number not for citation purposes 3 and anticoagulation were deemed necessary. Though anticoagulation Conclusion is considered useful in pregnant cyanotic patients on bed rest, there is no consensus on this issue [4]. The course and outcome of this patient with unrepaired VSD PA MAPCA illustrates the importance of teamwork and collaboration Between the 28th and 34th weeks, there was concern for the mothers among departments in resource-poor settings lacking a dedicated worsening hypoxemia, increasing hematocrit and deteriorating effort ACHD unit as the preferred care facility. In pregnant patients with tolerance. Supplemental oxygen was administered and close fetal functionally single ventricle, clinicians should expect a decreasing monitoring observed. The planned mode and timing of delivery for systemic vascular resistance favoring enhanced right-to-left shunting this patient was vaginal delivery under epidural analgesia at term. This and worsening cyanosis in the third trimester. In the immediate post- had to be revised when oligohydramnios became evident at 34 weeks partum period, a combination of increased systemic vascular gestation. The choice of anesthetic technique is important considering resistance and a large increase in venous return may lead to the profound effects anesthetic agents have on systemic vascular decompensated congestive heart failure. Close surveillance during resistance and the implications for pulmonary blood flow and oxygen pregnancy, delivery and the puerperium is essential in ensuring a saturation in this patient. The hemodynamic goals during anesthesia favorable outcome. were to maintain sinus rhythm, keep the systolic blood pressure above 100mmHg (or mean above 70mmHg) and prevent fluctuations beyond 20% of baseline. Suspected decreases in SVR were treated with norepinephrine by infusion. Although a slowly titrated epidural Competing interests anesthetic was initially contemplated, issues with prior anticoagulation swung the decision towards general anesthesia. Spinal anesthesia was The authors declare no competing interests. not favored because of the potential for rapid onset of vasodilatation and consequent increase in right-to-left shunting with worsening cyanosis. Authors’ contributions The usual hemodynamic changes occurring after delivery can prove DD-G conceived the idea for the paper, performed literature search, hazardous for patients with functionally single ventricle physiology. and contributed to drafting and reviewing the manuscript. EA Following delivery, extrusion of blood from the contracted uterus and contributed to reviewing and editing the manuscript. SO reviewed and decompression of the inferior vena cava results in a massive increase edited the manuscript. EO-A reviewed and edited the manuscript. FE in venous return. Furthermore, contraction of the utero-placental contributed to literature review, manuscript writing, editing, and vascular bed following delivery abruptly increases systemic vascular review. All authors read and approved the final manuscript. resistance. These hemodynamic changes may increase the cardiac output by as much as 80% [4] in the normal biventricular circulation connected in series. For the patient with functionally single ventricle physiology, these hemodynamic changes may be poorly tolerated. Figure Ostensibly, our patient's circulation could not cope with the sudden combined increases in venous return and systemic vascular return; she developed heart failure as a result, prolonging her intensive care Figure 1: (A) biventricular origin of the dilated ascending aorta (45 unit stay by seven days. Our experience emphasizes the point that mm) with 50% overriding; (B) MAPCA supplying right lung originates close attention in the intensive care unit is required to manage the from the descending aorta and passing posterior to trachea and dysfunctional peripartum physiology of patients with functionally esophagus (panel C) to reach the right side; (C) MAPCA to right lung single ventricle. passing posterior to trachea and esophagus. MAPCA to left lung demonstrates severe origin stenosis Page number not for citation purposes 4 References 6. Cabalka AK, Edwards WD, Dearani JA. Truncus arterioles. In Allen HD editor. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 1. Jacobs M, Mayer JE. Congenital heart surgery nomenclature and Philadelphia: Lippincott Williams & Wilkins, 7th ed, 2008. p. 912. database project: single ventricle. March 2000; 69(3): supplement: S197-S204. Google Scholar 7. Neumayer U, Somerville J. 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