DISCUSSION
Congenital heart defects like patent ductus arteriosus cause left-to-right shunts. This shunt causes the pulmonary vasculature to undergo pathological changes such as hypertrophy and fibrosis, consequently causing PAH. Initially, most patients are asymptomatic until a significant rise in pulmonary arterial pressure occurs and is thus present in adulthood, as seen in this patient [7]. Exercise intolerance, palpitations, and arrhythmias are all different presentations of PAH. Hemoptysis is a rare presentation and is seen in only 12% of adult survivors, as seen in this case [8]. These presentations are more pronounced in the presence of an additional shunt. As noted in this patient, there is another shunt between the right pulmonary artery and the aorta. To the best of our knowledge, this is the first anatomic variant of PDA reported and thus warrants a very patient-centered approach concerning its management.
The workup for hemoptysis invariably depends on the past medical history, which in our case was not significant despite the excessive pulmonary hypertension (68 mmHg on echocardiography) seen in this patient and is a rather rare clinical picture [9]. Chest X-ray (CXR) remains the best initial test of choice [10]. It determines the site of bleeding in 45 to 65% of the cases and the cause in 25 to 35% [11]. The next step has been a subject of debate as to whether bronchoscopy is better than CT in determining the cause of hemoptysis; however, recent studies demonstrate the superiority of CT [9]. In a case of active hemoptysis, such as ours, bronchoscopy can stimulate coughing and therefore increase the risk of bleeding. For this reason, it is often safer to delay the bronchoscopy until a definitive diagnosis is made [9]. A radiologic definition based on CT states that pulmonary artery hypertension is diagnosed when the pulmonary artery diameter is more than 28mm [11]. In our case, this is conclusively proven by a pulmonary artery diameter of 38mm [14]. The utilization of computed tomography (CT) in the assessment of patients with pulmonary hypertension can be advantageous in several ways by providing valuable insights into the potential underlying causes of the condition, such as lung parenchymal disease or primary cardiac processes, as well as chronic thromboembolic disease, and can effectively depict the current condition of the right ventricle, which is a crucial aspect of patient care in pulmonary hypertension cases. Echocardiography is used to consider the probability of PAH, and its parameters help to determine survival outcomes [13].
Embolotherapy is a widely accepted treatment modality for patients with severe recurrent hemoptysis [8]. Embolotherapy has been proven to show immediate control of bleeding in up to 90% of patients; however, it is associated with a significant risk of re-bleeding in approximately 50% of patients in long-term follow-up [12]. Recent studies have shown that acute presentations, like in this case if treated surgically, present with more complications in the long term [11]. Studies have shown that a surgical approach is also associated with high operative mortality (> 15%) [8]. On the contrary, conservative management has shown better patient outcomes in some patients [11]. This includes, a combination of cough suppressants, anti-fibrinolyitcs like tranexemic acid and antibiotics. Studies have shown a significant reduction in volume and duration of hemoptysis with the use of antifibrinolytic agents, thus reducing morbitdity[6]. The other spectrum of management is to control and adequately monitor PAH, which is the major cause of mortality in patients with multiple left to right shunts.
As one of the highest concentrations of PDE5 is found in the lungs, the PDE-5 inhibitor tadalafil was shown to effectively control PAH when given once a day [15] [16]. Furthermore, it is regarded as the most economical choice when compared to alternative treatment methods [17]. Hence conclusively we state that, due to the rare anatomic variant, as well as pronounced symptoms present in this patient, it might prove counter-intuitive to plan a surgical approach and have preferentially selected the aforementioned conservative approach.