Tabla 1. Right heart cardiac catheterization.

Figura 1. Parasternal view, short axis at papillary muscles level with evidence of right ventricular...

Figura 2. 4-chamber apical view showing significant right chamber dilatation and left chamber compr...

Figura 3. Amplatz guidewire in left superior pulmonary vein and 10 mm x 40 mm balloon with septal r...

Figura 4. Proper position and balloon opening in the septal plane.

Figura 5. Postoperative follow-up, subcostal 4-chamber view on the Doppler echocardiography showing ...

Introduction

Atrial septostomy (AS) was first described by Rashkind back in 1966 as a novel technique for palliative care of congenital disorders like large vessel transposition.¹ The objective was to create a solution of continuity in the interatrial septum. However, it would not be until 1983 when Rich and Lam performed this procedure for the first time to treat a patient with refractory pulmonary hypertension.² Such procedure has barely been described in the medical literature available and is performed in adult patients at the cath lab very rarely.

Case report

This is the case of a 20-year-old woman without known risk factors and a past medical history of mixed connective tissue disease diagnosed in 2018, Raynaud syndrome, Hashimoto’s thyroiditis, and bronchiectasis. She presents with clinical signs of abdominal pain, nausea, and vomiting of 1-week evolution. Vital signs at admission are within normal parameters. There is presence of abdominal distension, jugular ingurgitation 3/3, painful hepatosplenomegaly, systolic heart murmur 2/6 in tricuspid focus and mesocardium. The Doppler echocardiogram reveals the presence of severe right chamber dilatation, ventricular and atrial septum excursion towards the left due to pressure and volume overload, severe tricuspid regurgitation, and pulmonary systolic pressure of 85 mmHg estimated on the Doppler echocardiogram (Figure 1 and figure 2). The patient is, then, examined in the cardiology unit with clinical suspicion of right decompensated heart failure, and admitted to the cardiology unit. Targeted therapy is administered, and the patient is examined at the rheumatology unit with high diagnostic suspicion of SLE. The right heart cardiac catheterization performed (Table 1) confirms the diagnosis of pulmonary hypertension. The patient disease progression is poor with sustained hypotension. She is then transferred to the coronary care unit with signs of oliguria and shock while remaining unresponsive to vasoactive drugs. The Doppler echocardiogram confirms the presence of left chamber compression and a reduced cardiac output. Therefore, an emergency atrial septostomy is decided. Procedure is performed under neuroleptoanesthesia. A 5-Fr valved introducer sheath is inserted via right radial access with continuous invasive blood pressure monitoring followed by 8-Fr introducer sheath insertion via right venous femoral access. A 0.35 in guidewire was inserted and moved upwards towards the innominate vein. Afterwards, a transseptal puncture kit was advanced. A dedicated technique was used via transseptal puncture, and the right atrium was accessed. Heparinization was performed and an Amplatz guidewire was placed inside the left superior pulmonary vein. Balloon is then ascended towards the septal plane (figure 3) with successful opening (figure 4). Higher left ventricular end-diastolic pressure and far less arterial saturation are confirmed, which is the moment when it was decided to finish the procedure. Patient progression revealed an immediate hemodynamic improvement, and pulmonary vasodilation. Afterwards, the patient was referred to a center specialized in pulmonary hypertension and cardiac transplant where the patient achieved clinical stability. Optimal medical therapy was prescribed, and she entered a program of cardiopulmonary transplantation.

Discussion

Interventional therapy with percutaneous AS in the adult patient is extremely rare. No more than 300 cases have been reported in contemporary case series in specialized centers, and clinical guidelines on pulmonary hypertension only recommend this procedure on rare clinical occasions.³

Different technical variations of this procedure have come up since its inception like knife or Park AS,⁴ the use of stenting,⁵ and the stepped approach for balloon inflation.⁶

In the case described here, one single balloon was used given the patient’s refractory status. Procedural results were excellent.

The mechanism through which the procedure seems more beneficial would be flow shunting, right chamber pressure reduction, and left chamber pressure increase to reach increased cardiac output and reduced systemic O2 saturation. Mortality rate described is 7.1% within the first 24 hours and 14.8% at 1 month. The most relevant variable is performing the procedure in experienced centers and avoiding it in the presence of poor prognostic indicators (right atrial pressure > 20 mmHg, PCWP > 18 mmHg, severe RV failure, and SO2 < 90%).⁶ There seems to be a lower mortality rate (2% at 1 month) in today’s procedures thanks to the balloon stepped approach,⁷ and the use of fusion imaging.⁵,⁸ In some selected cases and as bridging or bailout therapy, it seems like the treatment of choice when the remaining therapies have failed or are not available.

Conclusion

In this case, septostomy as a bailout procedure brought immediate clinical benefits to the patient and no complications at 2-month follow-up.

This procedure should always be carefully assessed and performed by the heart team including specialists in pulmonary hypertension and heart transplant. Left-to-right or right-to-left shunt seems to be a developing field of research that will bring new tools to treat our patients when properly indicated and supported by scientific evidence.

1. Rashkind WJ, Miller WW. Creation of an atrial septal defect without thoracotomy. A palliative approach to complete transposition of the great arteries. JAMA. 1966;196:173.

2. Rich S, Lam W. Atrial septostomy as palliative therapy for refractory primary pulmonary hypertension. Am J Cardiol. 1983;51:1560–1561

3. Keogh A, Mayer E, Benza R, et al. Interventional and Surgical Modalities of Treatment in Pulmonary Hypertension. J Am CollCardiol. 2009 Jun, 54

4. Park SC, Neches WH, Mullins CE, et al. Blade Atrial Septostomy: Collaborative Study. Circulation. 1982;66:258

5. Prieto LR, Latson LA, Jennings C. Atrial septostomy using a butterfly stent in a patient with severe pulmonary arterial hypertension. CatheterCardiovascInterv. 2006;68:642–647.

6. Sandoval J, Gaspar J, Peñas H, Santos LE, Córdova J, del Valle K, et al. Effects of atrial septostomy on the survival of patients with severe pulmonary arterial hypertension. EurRespir J 2011; 38:1343-48.

7. Khan MS, Memon MM, Amin E, et al. Use of Balloon Atrial Septostomy in Patients With Advanced Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis. Chest. 2019;156(1):53-63. doi:10.1016/j.chest.2019.03.003

8. Moscussi M, Dairywala IT, Chetcuti S, et al. Balloon atrial septostomy in end-stage pulmonary hypertension guided a novel intracardiac echocardiographic transducer. Catheter CardiovascInterv. 2001;52:530–534.

Para descargar el PDF del artículo
Emergency atrial septostomy in severe pulmonary hypertension and shock

Haga click aquí