Pulmonary hypertension crisis management in adult atrial septal defect surgical closure: A case report

Prieta Adriane; Rudyanto Sedono; Ni Luh Kusuma Dewi

doi:10.4103/bjoa.bjoa_97_22

CASE REPORT

Year : 2022 | Volume : 6 | Issue : 4 | Page : 251-253

Pulmonary hypertension crisis management in adult atrial septal defect surgical closure: A case report

Prieta Adriane¹, Rudyanto Sedono¹, Ni Luh Kusuma Dewi²
¹ Department of Anesthesiology and Intensive Therapy, Medical Faculty, University of Indonesia, Jakarta, Indonesia
² Department of Anesthesiology and Intensive Therapy, Harapan Kita National Cardiovascular Hospital, Jakarta, Indonesia

Date of Submission	25-Mar-2022
Date of Decision	09-May-2022
Date of Acceptance	17-May-2022
Date of Web Publication	31-Oct-2022

Correspondence Address:
Prieta Adriane
Department of Anesthesiology and Intensive Therapy, Medical Faculty, University of Indonesia, Sudirman Park Apartment B 02 CD Jl. KH Mas Mansyur kav.35, Jakarta 10220
Indonesia

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/bjoa.bjoa_97_22

Abstract

Atrial Septal Defect (ASD) is the most common congenital disorder found in adulthood, accounting for 35% of Adult Congenital Heart Disease (ACHD). In ASD, there is a defect in the septum/wall between right and left atria, resulting in left-to-right shunt which lead to an increase of the right heart volume and pulmonary circulation. If left untreated, increased pulmonary resistance develop to pulmonary hypertension (PH) which results in a progressive decrease in right ventricular function, causing right heart failure and death. Although ASD surgical closure is not a complicated procedure, patients with complication of PH and right heart failure have a high mortality rate if not managed properly. Increase in mortality was mainly due to perioperative pulmonary hypertensive crisis (PH crisis), condition of an acute increase in mean pulmonary arterial pressure (mPAP) exceeding mean systemic arterial pressure (MAP). In this case report, we will discuss the management of postoperative adult patients with ASD closure complicated by intraoperative pulmonary hypertensive crisis who are treated in the intensive care unit (ICU).

Keywords: Atrial septal defect, ICU management, PH crisis, pulmonary hypertension

How to cite this article:
Adriane P, Sedono R, Dewi NL. Pulmonary hypertension crisis management in adult atrial septal defect surgical closure: A case report. Bali J Anaesthesiol 2022;6:251-3

How to cite this URL:
Adriane P, Sedono R, Dewi NL. Pulmonary hypertension crisis management in adult atrial septal defect surgical closure: A case report. Bali J Anaesthesiol [serial online] 2022 [cited 2023 Mar 22];6:251-3. Available from: https://www.bjoaonline.com/text.asp?2022/6/4/251/359935

Introduction

Prevalence of Atrial Septal Defect (ASD) is 1.6 per 1000 births, with 97% of patients are likely to survive into adulthood. In ASD, there is a defect in septum between the right and left atria, resulting in left to right shunt causing an increase in the right heart volume and pulmonary circulation. Increased pulmonary resistance can lead to pulmonary hypertension (PH) resulting in a progressive decrease in right ventricular function, causing right heart failure and mortality. Patients with significant shunt which cause right ventricular volume overload are indicated for ASD closure.^[1]

PH prevalence after ASD closure in adult patients varied from 5 to 50%. Although ASD surgical closure is not a complicated procedure, patients with complicated PH and right heart failure might be fatal if not treated properly. Increase in mortality mainly due to the high incidence of perioperative pulmonary hypertensive crises (PH crises), which is the mean pulmonary arterial pressure (mPAP) increased acutely beyond the mean systemic arterial pressure (MAP).^[2],[3] In this case report, we will discuss the management of postoperative adult patients with ASD closure complicated by PH crisis in the intensive care unit.

Case Report

A 29-year-old woman complaining a shortness of breath and fatigue since her first pregnancy in 2015. After giving birth, the patient was diagnosed with ASD and advised for surgery but she refused at that time. Patient had a history of two spontaneous abortions after being diagnosed. Patient had undergone 6MWT and Right Heart Catheterization (RHC) tests before it was decided for ASD closure surgery with final diagnosed as of ASD L-R shunt, high flow, low resistance, and high probability of PH. Patient given furosemide 1 × 40 mg, ramipril 1 × 1.25 mg, sildenafil 3 × 25 mg, and spironolactone 1 × 25 mg orally one-month prior surgery.

The patient was admitted to the hospital one day before the surgery. On physical examination, vital signs were within normal limits, with peripheral oxygen saturation (SpO2) was 94% on room air. Remarkable physical examinations were audible murmur at ICS 4 parasternal line. Chest X-ray showed cardiomegaly, cardiothoracic ratio >50% [Figure 1]. Echocardiography showed; ASD secundum is difficult to close with a device because the posterior and inferior rims are thin; mild mitral regurgitation due to AML prolapse (A3); severe tricuspid regurgitation with probable PH.

Figure 1: Chest radiology; (1) Pre-operative, (2) Direct post-operative, (3) Post-extubation

Click here to view

Perioperatively, induction of anaesthesia went smooth and maintenance was given continuously. Preoperative TEE (Transoesophageal Echocardiography) showed that RA, RV dilated, LV small, IVS pushed to the left (D-shaped), large secundum ASD 3.5 cm, L to R shunt, TR moderate, tricuspid annulus 3.5 cm, trivial MR, pulmonary artery size twice from the aorta (AO: 1.97 cm, PA: 4.4 cm). Surgical procedures performed were ASD closure with a pericardial patch, patent foramen ovale (PFO) creation, and repair of the tricuspid valve with Kay procedure. Cardiopulmonary bypass (CPB) duration was 47 minutes, aortic clamping time (AOX) was 23 minutes. After CPB weaning, mPAP exceeded the MAP, it was decided to administer nitric oxide (NO) inhalation in the operating room. Patient hemodynamic when discharged from the operating room: arterial blood pressure 94/48 (63) mmHg, heart rate 81 beats/minute, sinus rhythm, central venous pressure 7–8 mmHg, PAP 74/50 (58) mmHg, with adrenaline support 0.07 mcg /kg/min, NTG 3 mcg/kg/min, milrinone 0.5 mcg/kg/min and inhalation NO 20 ppm.

Postoperatively, the patient was admitted to surgical intensive care unit (ICU) completely sedated and under influence of muscle relaxant, mechanical ventilation done with iNO 20 ppm in the circuit. First day in ICU, iNO was increased to 30 ppm due to oversystemic mPAP, low cardiac output, metabolic acidosis, and hypercarbia. Diuresis was enhanced with 20 mg/hour furosemide drip. Patient was given an initial dose of sildenafil 3 × 3.125 mg from nasogastric tube (NGT) and the dose was increased gradually. iNO was reduced to 20 ppm then Iloprost was administered with dose of 8 × 5 mcg inhalation on second day. iNO was weaned and then discontinued. On the fourth day, we stopped sedation, then patient was awakened, and started weaning from mechanical ventilation. After adequate spontaneous breathing achieved, patient was extubated.

Patient was transferred from ICU to intermediate ward fully conscious, good contact and motor skills, adequate spontaneous breathing with saturation 98–99%. Hemodynamic patient is stable with diuresis >1 cc/kg/hour with iloprost 8 × 5 mcg and sildenafil 3 × 25 mg PO. The patient was discharged from the hospital on the 8th postoperative day.

Discussion

Patients with isolated ASD are usually asymptomatic, even with large left-to-right shunt do not develop obvious symptoms until adulthood. Incidental diagnosis sometimes due to the presence of a heart murmur or an abnormal radiograph or ECG. Pregnancy will lead to a firm diagnosis because heart murmur becomes audible. Patients with significant shunts complains of fatigue, palpitations, activities intolerance, shortness of breath, and syncope. Pulmonary arterial hypertension (PAH) occur in response to pulmonary circulation chronic volume overload, caused by a left-to-right shunt which lead to change of the pulmonary vasculatures. PH Hemodynamic definition is condition which mPAP >25 mmHg at rest or >30 mmHg with activity.^[4],[5]

PH crisis is a clinical condition that occurs when the mPAP exceeds the MAP. Perioperative PH crises incidence is estimated to range between 2% and 5%, appearing to be more related with CPB use. Causative mechanism thought to be sudden pulmonary vasoconstriction, leading to right ventricular failure and systemic circulatory hypotension, which resulting severe tissue hypoxia and eventually death. PH crisis also can be contributed by endothelial cell injury, systemic inflammatory response, inhibition of nitric oxide (NO) synthesis, and elevated endothelin.^[3],[6]

Important management of a PH crisis post cardiac surgery includes: (1) acute intervention to compensate for tissue hypoxia and extreme acidosis, and (2) good assessment and treatment of right heart failure. Right ventricular (RV) failure is the most common cause of death in PH patients, whilst RV function is a major determinant of morbidity and mortality in this population. In patients with pulmonary arterial hypertension (PAH), right ventricular failure with low cardiac output and high RV filling pressure is commonly seen. PH crisis requires aggressive combination therapy, and carefully administer fluids carefully, manage inotropes and vasopressors, maintain normal heart rhythm and prevent pain with adequate analgesics.^[7]

PAH-specific vasodilators should be considered if the general therapies do not improve hemodynamic deterioration and hypoxia. Administer drugs, such as endothelin receptor antagonists (ERA), phosphodiesterase-5 (PDE-5) inhibitors, and prostacyclin analogues, led to significant increase of life expectancy surgery for adult congenital heart lesions.^[3],[8]

In post-CPB PH crisis patients, causes of sudden pulmonary vasoconstriction are protamine administration, and systemic inflammatory reaction syndrome (SIRS) triggered by CPB. Drugs used to treat an intraoperative PH crisis in this patient were intravenous milrinone at a dose of up to 0.5 mic/kg/minute, nitroglycerine 3 mic/kg/minute and adrenaline 0.07 mic/kg/minute, with combination of inhaled nitric oxide 20 ppm.^[3]

Milrinone is a PDE-3 phosphodiesterase inhibitor, used extensively to reduce PVR in cardiac surgery, and induce cardiac inotropy, but can cause systemic hypotension will increase the need for vasoactive drugs. Nitroglycerine acts as a vasodilator and functions to reduce right heart preload and improve cardiac oxygen consumption. Adrenaline helps contractility, especially the right heart, to overcome acute increase in PVR during PH crisis.^[8],[9]

Inhaled NO resulting in selective pulmonary vasodilation by activating the guanosine monophosphate (cGMP) cycle signalling pathway in pulmonary vascular smooth muscle cells. iNO has become standard therapy for postoperative PAH control and PH crisis, as recommended by European guidelines in 2004. However, the dose and duration of iNO administration between institutions varies. In general, the first-line therapy for perioperative refractory PAH is iNO. If ineffective, the use of extracorporeal membrane oxygenation (ECMO) as modality should be considered. iNO dose is usually started at 5–20 ppm, and can be uptitrated to maximum of 80 ppm, however it is important to give the lowest effective dose, in order to minimize toxicity.^[9]

PDE-5 inhibitor, such as sildenafil, has been shown effective as part of the treatment postoperative PAH and can be useful as an adjunct therapy to facilitate weaning from intravenous and inhaled pulmonary vasodilators. As recommended treatment for PAH, Oral sildenafil may reduce the incidence of rebound PH after discontinuation of iNO in postoperative period. In this patient, sildenafil therapy was started 1 month before surgery, and was given immediately postoperatively in the ICU with a small dose that was increased gradually via NGT then continued orally^[10]

Conclusion

Some cases of severe ASD where PH has occurred and the possibility of right heart failure, there are several things that must be considered, including the administration of therapy preoperatively to reduce high PVR, the selection of the correct procedure, and postoperative multi-modalities in the case of PH crisis to improves cardiac output and prevents PH rebound or re-PH crisis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Post MC. Association between pulmonary hypertension and an atrial septal defect. Neth Heart J 2013;21:331-2.
2.	Zwijnenburg RD, Baggen VJM, Witsenburg M, Boersma E, Roos-Hesselink JW, van den Bosch AE. Risk factors for pulmonary hypertension in adults after atrial septal defect closure. Am J Cardiol 2019;123:1336-42.
3.	Brunner N, de Jesus Perez VA, Richter A, Haddad F, Denault A, Rojas V, et al. Perioperative pharmacological management of pulmonary hypertensive crisis during congenital heart surgery. Pulm Circ 2014;4:10-24.
4.	Nashat H, Montanaro C, Li W, Kempny A, Wort SJ, Dimopoulos K, et al. Atrial septal defects and pulmonary arterial hypertension. J Thorac Dis 2018;10:S2953-S65.
5.	Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, et al; ESC Scientific Document Group. 2020 Esc guidelines for the management of adult congenital heart disease. Eur Heart J 2021;42:563-645.
6.	Jain S, Dalvi B. Atrial septal defect with pulmonary hypertension: When/how can we consider closure? J Thorac Dis 2018;10(Suppl 24):S2890-S8.
7.	Hoeper MM, Granton J. Intensive care unit management of patients with severe pulmonary hypertension and right heart failure. Am J Respir Crit Care Med 2011;184:1114-24.
8.	Olschewski H. Inhaled iloprost for the treatment of pulmonary hypertension. Eur Respir Rev 2009;18:29-34.
9.	Levine AB, Punihaole D, Levine TB. Characterization of the role of nitric oxide and its clinical applications. Cardiology 2012;122:55-68.
10.	Michelakis E, Tymchak W, Lien D, Webster L, Hashimoto K, Archer S. Oral sildenafil is an effective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension: Comparison with inhaled nitric oxide. Circulation 2002;105:2398-403.